PRIMARY EYECARE CLINIC ···· ····················································································· ·············· ·· ····························································· ··················································································································•···········

c£ . The Photic Sneeze Response: NO'' · wt~'fBE AD . t• R f ,.H,s ~f.\,-e.R\'"'~oP'lf\\G\{r escr1p 1ve eport o

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The prevalence of the photic sneeze response (PSR) as well as other characteristics have been studied in selected pop­ulations. However, the PSR has not been investigated in a general eye care patient population. This study was per­formed in an attempt to characterize the epidemiologic, descriptive, and demographic aspects of the photic sneeze response among patients attending for primary eye care.

· ·•·• · ··· •·•··•· · •oo•• • •• "·' "' '''' ' ' '''''''''' '''' '''''' ' ''' ''''' '' 'M E T H 0 0 5

A questionnaire on demographics , risk factors, and trigger­ing stimuli was distributed to 500 consecutive patients pre­senting for a general eye examination at an academic health center optometry clinic.

. .......... . .. . .... . .... . .... .. . . .. .. ................ . .. . .... .. .... . .. RESULTS

Three hundred and sixty-seven of 500 questionnaires were returned (73.4%). Among this sample 33% were self-recog· nized photic sneezers with the majority being females (67%) and Caucasian (94.3%). Statistically significant correlations were found between the presence of photic sneezing and the presence of a deviated nasal septum and a non-signifi­cant association was found with tobacco use. Uniform fre­quency of sneezing does not occur in response to light stim­ulus; only 12.3% of sneezers responded consistently to sun­light exposure. The majority of sneezers (90.7%) responded with three or fewer sneezes. The interval between succes· sive sneezes was fewer than 19 seconds in 85% of respon­dents. Fewer than 27% of respondents were able to recall a parent who exhibited a sneeze response.

The PSR is not an uncommon phenomenon. Systemic asso­ciations with the PSR are as diverse as deviated nasal sep­tum and tobacco use. Results suggest that there may be a threshold level of light or frequency of light exposure which produces the response and that more patients may acquire the response than inherit it.

Photic sneeze response, solar sneeze reflex, general eye population

Semes LP. Amos JF. Waterbor JW. The photic sneeze response: a descriptive report of a clinic population. J Am Optom Assoc 1995; 66(6):372-7.

372

Leo P. Semes, 0. D.

John F. Amos, O.D., M.S.

John W. Waterbor, M.D., Dr. P. H.

The photic sneeze response (PSR) was first reported by Watson' in 1875. He suggested that the responsible mechanism was morbid (super) sensitivity of the trigeminal nerves. Other reports of non-photic stimuli have appeared,'4 but there has been a paucity of studies on photic stimuli. Of subsequent reports, only five studies conducted surveys to ascertain prevalence or descriptive char­acteristics.s-•o The PSR has not been investigated in a patient population presenting for routine eye care.

In 1954 Sedan" reported the first clinical cases since Watson's description of PSR more than 75 years earlier. He described six cases in which patients responded with a sneeze response to the light of an ophthalmoscope, the sun, flash photography, or an ultraviolet source. Sedan considered this response an uncommon phenomenon. Everett5 was the first to investigate the PSR by survey; he interviewed 75 hospital employees and queried 343 medical students and neurology residents by means of a questionnaire.' His findings indicated that the prevalence of self-observed photic sneezing was higher among white males (27.8%) than any other race or gender group. Prevalence of the photic sneeze response was also reported to be higher among individuals who had noted the "reflex" among family members. Everett postulated that parasympathetic excitation resulting in reflex rhinitis may be the physiologic mechanism responsible for the PSR. He also noted that stimulation of the eye through the optic nerve by light (creating a spillover of affer­ent impulses) or non-photic stimulation such as rubbing the eyes (trigeminal nerve stim­ulation) could produce a sneeze response.

Collie and colleagues6 reported on the inheritance pattern of four physicians who expe­rienced PSR from exposure to sunlight. Based on histories, all four of the affected kin­dreds revealed an autosomal dominant (AD) pattern with a high degree of penetrance. Beckman and Nordensen7 questioned 460 Swedish blood donors in regard to the PSR. They reported that 110 (24%) of this sample experienced a PSR response but without significant association with gender, and that only 18 pedigrees demonstrated an AD inheritance pattern. Peroutka and Peroutka8 found that 36% of 25 neurologists surveyed experienced PSR. They also reported on the PSR in one family in which the trait was passed from father to daughter, suggesting that the PSR is transmitted in an autosomal dominant fashion . Inconclusive evidence implicating the AD inheritance pattern is offered by Forrester,9 who surveyed 114 consecutive patients in a general surgery prac­tice. He found that 29 patients (25%) reported the presence of PSR. He also reported

.JOURNAL OF THE AMERICAN OPTOMETRIC ASSOCIATION VOLUME 66/NUMBER 6 / JUNE 95

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that among 15 couples with children in which one of the parents demonstrated the response, 17 of 3 7 (46%) children also manifested the PSR. While these data are sparse and perhaps incomplete in control­ling for outside influences, they appear to support the autosomal dominant mode of inheritance for the PSR. The question of an acquired variety or the possibility of another mode of genetic transmittance remains.

Lang and Howland10 surveyed rhinitis patients and reported a prevalence of 11% (15/138) . They also found a prevalence of 35% (50/144) photic sneezers among the staff of the Scripps Medical Group. Their data suggest no significant correlation between PSR and rhinitis or asthma.

Several additional reports of the association of PSR with various disorders have appeared."·" Morris'8 has explored_laboratory findings associated with the PSR, while Whitman and Packer'9 recently reviewed exist­ing studies in detail.

Because of the relatively large number of photic sneezers apparently present in the population, a study was undertaken to determine the prevalence of the PSR in an eye clinic population and to determine whether factors other than genetics are determinants in the photic sneeze response. In addition, this study sought to determine what physical features of the phe­nomenon were consistent among patients with PSR that would suggest it is an acquired rather than an inherited trait.

Methods A questionnaire was designed to determine the preva­lence of PSR and to elicit the descriptive factors asso­ciated with sneezing in response to light stimulation. Additionally, medical, social, and demographic infor­mation was solicited to determine if there were spe­cific correlations of PSR with factors cited in the liter­ature (e.g., deviated nasal septum).

The survey instrument was distributed to 500 consec­utive patients presenting to the UAB School of Optometry for general eye examination during a tO­week period from September to November 1991. A receptionist was available to answer any questions about the purpose of the survey and how the infor­mation gathered would be used. All patients to whom the questionnaire was given had the opportunity to decline to complete it.

*56 sneezers did not respond

Results Of the 500 questionnaires distributed, 367 (73.4%) were completed and returned. A "completed" ques­tionnaire contained responses to at least 90% of the questions (for sneezers) or to all questions in Part I (demographics, for non-sneezers). Of the 367 respondents, 122 (33.2%) were self-recognized photic sneezers (PSR group; study population) ; these individuals were used to determine the risk factors and family history which might influence the photic sneeze response.

The mean age of the PSR group was 45.8 years (range 12 to 89 years) . The mean age of the non-photic sneezers (245 respondents to the questionnaire who denied sneezing in response to light stimulation; con­trol group) was 44.0 years (range 13 to 77 years): The most prevalent age for the recognition of photic sneezing was the sixth decade (26/65; 40.0%).

ln addition to age and recognition of the PSR, the sur­vey instrument solicited demographic, medical, and

Table 2 373

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that among 15 couples with children in which one of the parents demonstrated the response, 17 of 37 (46%) children also manifested the PSR. While these data are sparse and perhaps incomplete in control­ling for outside influences, they appear to support the autosomal dominant mode of inheritance for the PSR. The question of an acquired variety or the possibility of another mode of genetic transmittance remains.

Lang and Howland 111 surveyed rhinitis patients and reported a prevalence of 11 % (15/138). They also found a prevalence of 35% (50/1 44) photic sneezers among the staff of the Scripps Medical Group. Their data suggest no significant correlation between PSR and rhinitis or asthma.

Several additional reports of the association of PSR with various disorders have appeared. "-'7 Morris" has explored laboratory findings associated with the PSR, while Whitman and Packer''' recently reviewed exist­ing studies in detail.

Because of the rclatiwly large number of photic sneezers apparently present in the population, a study was undertaken to determine the prevalence of the PSR in an eye clinic population and to determine whether factors other than genetics are determinants in the photic sneeze response. In addition, this study sought to determine what physical features of the phe­nomenon were consistent among patients with PSR that would suggest it is an acqui red rather than an inherited trait.

Methods A questionnaire was designed to determine the preva­lence of PSR and to elicit the descriptive factors asso­ciated with sneezing in response to light stimulation. Additionally, medical , social , and demographic infor­mation was solicited to determ ine if there were spe­cific correlations of PSR with factors cited in the li ter­anu·e (e.g., deviated nasal septum).

The survey instrument was distributed to 500 consec­utive patients presenting to the UAB School of Optometry for general eye examination dming a 10-week period from September to November 1991. A receptionist was available to answer any questions about the purpose of the survey and how the infor­mation gathered would be used. All patients to whom the questionnaire was given had the opportunity to decline to complete it.

JOUR N AL OF THE AMER I CAN OPTOME T RIC ASSOC I A T IO N

Results Of the 500 questionnaires distributed, 367 (73.4%) were completed and renm1ed. A "completed" ques­tionnaire contained responses to at least 90% of the questions (for sneezers) or to all questions in Part I (demographics, for non-sneezers) . Of the 367 respondents, 122 (33.2%) were self-recognized photic sneezers (PSR group; study population); these individuals were used to determine the risk factors and family history which might influence the photic sneeze response.

The mean age of the PSR group was 45.8 years (range 12 to 89 years). The mean age of the non-photic sneezers (245 respondents to the questionnaire who denied sneezing in response to light stimulation; con­trol group) was 44.0 years (r;mge 13 to 77 years). The most prevalent age fo r the recognition of photic sneezing was the sixth decade (26/65; 40.0%).

In addition to age and recognition of the PSR, the sur­vey instrument solicited demographic, medical, and

Table 2

not respond

37 3

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PRIMARY EYECARE CLINIC .......... ............................. ................................................. .. ..................................................................................................................................................... ............. ..

374

social information. Not all subjects answered every question. With respect to gender, of all females who completed the questionnaire, 39.1% reported photic sneezing; of all males, 30.0% responded positively, an insignificant difference (p=0.15) . With regard to race, the vast majority of respondents (301/367; 82.0%) was white. Of whites, 38.2% (115/301) reported experiencing the PSR, as compared to only 8.2% (5/61) of blacks (p<O.OOOOI).

The distribution of eye color varied greatly among self-recognized photic sneezers. Nearly as many blue­eyed (341122; 27.9%) as dark-brown eyed (38/122; 31.1%) photic sneezers were identified. The distribu­tion of eye color for sneezers and non-sneezers did not vary markedly (p=0.14). Of all eye colors, green was the color for which PSR prevalence was highest (48.6%).

Among significant medical history, questions were included concerning the coexistence of diabetes mel­litus, asthma, thyroid dysfunction, hypertension and allergy, as these associations have been previously reported.10

· ' 9 The distributions of sneezers and non­sneezers who had these conditions were similar. Forty-one of 122 (33.6%) photic sneezers responded positively to the presence of allergies. Interestingly, 71 of 245 (29.0%) control-group respondents also indi­cated a positive allergy history, making allergy the most prevalent condition identified in both groups.

Table 3

We inquired specifically about the presence of deviated nasal septum. All photic sneezers responded. Unfortunately, nearly half (60/122; 49.1 %) were unaware of the presence or absence of deviated nasal septum. Of the 62 remaining photic sneezers who were aware of the presence or absence of deviated nasal sep­tum/ (14.2%) indicated that it was present. Only 7 of 152 (4.6%) of the control group aware of the presence or absence of deviated nasal sep­tum indicated its presence. The higher prevalence of deviated

nasal septum in sneezers compared to non-sneezers (excluding unknowns) was significant (p=0.0007).

All respondents answered the question concerning tobacco use. Thirty-three of 122 (27.0%) photic

sneezers used tobacco products. Forty-six of 245 (18.8%) control-group respondents admitted to tobacco use. This difference did not achieve statistical significance at the 0.05 level.

Respondents exhibiting the PSR were asked to esti­mate the age at which photic sneezing was first observed. Of the 66 photic sneezers who responded with an estimate, the second decade was the most prevalent. By age 30, over three-quarters (87.8%) of the PSR group had recognized the phenomenon (Table 1) .

In an attempt to determine the consistency \vith which exposure to sunlight produced a sneeze response, respondents were queried concerning frequency of sneezing. All122 of the PSR group responded to this question. Over half (63/122; 51.6%) of the group indicated that only once in a while did sunlight pro­duce the response. Fifteen (12.3%) respondents indi­cated that sunlight produced a sneeze response almost every time (Table 2).

The number of successive sneezes was limited to three or fewer in over 90% of cases. The frequency of sneezing in response to sunlight was 1 or 2 sneezes in two-thirds of cases. Sneezing frequency dropped off rapidly after three successive sneezes (Table 3).

Forty repeat photic sneezers were able to offer a determination of the latency between sneezes. 1\venty­four (60.0%) indicated that the interval between suc­cessive sneezes was less than 10 sec. Ten respondents (25%) indicated an interval of 10 to 19 sec. Only 6 (15%) of repeat photic sneezers indicated a latency of greater than 20 sec (Table 4).

The seasonal influence on photic sneezing was incon­sequential among 59 of 117 (50.4%) photic sneez­ers. Of those reporting a seasonal difference, the peak season for sneezing was nearly equally divided between spring (19.7%) and summer (21.4%). Although the degree of brightness did not matter to 2 7 of 106 (25.7%) photic sneezers, 78 of 106 sneezers (73.6%) indicated that clear days were more likely to produce the response than cloudy days. Did sneezing occur when the intense light stimulus was \vithdrawn? An overwhelming majority of photic sneezers did not notice a sneeze response when leaving the stimulating environment. That is to say, 101 of Ill respondents (91.1 %) indicated that they did not sneeze when leav­ing sunlight. Reported effects accompanying the phot­ic sneeze response included lacrimation (55.6% of those responding to the question) , squinting

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PRIMARY EYECARE CLI NI C

social information. Not all subj ects answered every question. With respect to gender, of ali femal es who completed the questionnaire, 39.1 % reported photic sneezing; of all males, 30.0% responded positively, an insignificant difference (p=0.15). With regard to race, the vast majority of respondents (30ll367; 82 .0%) was white. Of whites, 38.2% (115/301) reported experiencing the PSR, as compared to only 8.2% (5/61) of blacks (p<O.OOOOI).

The distribution of eye color varied greatly ~unong self-recognized photic sneezers. Nearly as m~my blue­eyed (341122; 27.9%) as dark-brown eyed (38/1 22; 31.1 %) photic sneezers were identified. The distribu­tion of eye color for sneezers and non-sneezers did not vaty markedly (p=0. 14). Of all eye colors, green was the color for which PSR prevalence was highest (48.6%).

Among signifiGmt medical history, questions were included concerning the coexistence of diabetes mel­litus, asthma, thyroid dysfunction , hypertension and allergy, as these associations have been previously reported . " '·~~ The distributions of sneezers and non­sneezers who had these conditions were similar. Forty-one of 122 (33.6%) photic sneezers responded positively to the presence of allergies. In terestingly, 7 I of 245 (29.0%) control-group respondents also indi­cated a positive allergy history, making allergy the most prevalent condition identified in both groups.

Number of successive sneezes caused by sunlight

We inquired specifically about the presence of deviated nasal septum. All photic sneezers responded. Unfortunately, ne~·ly half (60/122; 49. 1%) were unaware of the presence or absence of deviated nasal septum. Of the 62 remaining photic sneezers who were aware of the presence or absence of deviated nasal sep­tum ,'~ (14.2%) indicated that it was present. Only 7 of 152 (4.6%) of the control group aware of the presence or absence of deviated nasal sep­tum indicated its presence. The

sneezes number* %

*4 sneezers did not respond

374

Table 3 higher prevalence of deviated

nasal septum in sneezers compared to non-sneezers (excluding unknowns) was significant (p=0.0007) .

All respondents answered the question concerning tobacco use. Thirty-three of 122 (27.0%) photic

.JOURNAL OF THE AM ER ICAN OPTOMETRIC ASSOCIATION

--- ~ - -~ -- -

sneezers used tobacco products. Forty-six of 245 (18.8%) control-group respondents admitted to tobacco use. This difference did not achieve statistical significance at the 0.05 level.

Respondents exhibiting the PSH were asked to esti­mate the age at which photic sneezing was first observed. Of the 66 photic sneezers who responded with an estimate, the second decade was the most prevalent. By age 30, over three-quarters (87.8%) of the PSH group had recognized the phenomenon (Table 1).

In an attempt to determine the consistency with which exposure to sunlight produced a sneeze response, respondents were queri ed concerning frequency of sneezing. All 122 of the PSR group responded to this question. Over half (63/122; 51.6%) of the group inclicated that only once in a whi le did sunlight pro­duce the response. Fifteen ( 12.3%) respondents incli ­cated that sunlight produced a sneeze response almost eve1y time (Table 2 ) .

The number of successive sneezes was limited to three or fewer in over 90% of cases. The frequency of sneezing in response to sunlight was I or 2 sneezes in two-thirds of cases. Sneezing frequency dropped off rapidly after three successive sneezes (Table 3).

Forty repeat photic sneezcrs were able to offer a determination of the latency between sneezes. '1\venty­four (60.0%) indicated that the interval between suc­cessive sneezes was less than I 0 sec. Ten respondents (25%) indicated an interval of I 0 to 19 sec. Only 6 05%) of repeat photic sneeze rs indicated a latency of greater than 20 sec (Table 4).

The seasonal influence on photic sneezing was incon­sequential among 59 of 11 7 (50.4%) photic sneez­ers. Of those reporting a seasonal difference, the peak season for sneezing was nearly equally divided between spring (19.7%) and su mmer (2 1.4%). Although the degree of brightness did not matter to 27 of 106 (25.7%) photic sneezers, 7R of 106 sneezers (73.6%) indicated that clear clays were more likely to produce the response than cloudy days. Did sneezing occur when the intense light stimulus was withdrawn? An overwhelming majority of photic sneezers did not notice a sneeze response when leaving the stimulating environment. That is to say, lO 1 of 111 respondents (91 .1 %) indicated that they did not sneeze when leav­ing sunlight. Reported effects accompanying the phot­ic sneeze response included lacrimation (55.6% of those responding to the question) , squinting

VOLUM E 66 / NUMBER 6 / .JUNE 95

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(64.7%), and yawning (14.4%) .

With regard to evidence for a genetic transmittance pattern of PSR, 48 of 122 PSR respondents (39.3%)

Average number of seconds between successive sneezes

caused by sunlight, among sneezers

*39 repeat sneezers did not respond

Table 4 reported that no direct relatives were also photic sneezers. In fact, only 21 of 79 photic sneezers (17.2%) indicated that either parent experienced the photic sneeze response.

Other stimuli to sneezing identified in the question­naire included automobile headlights, lamps in doc­tors' offices, and pollen. Eighty-six of 122 sneezers (70.5%) reported that pollen was a stimulus to sneez­ing. Thirty-one of 122 (25.4%) reported that head­lights or floodlights also prompted a sneezing response.

Discussion Sneezing in response to light has various names. Terms such as the photo-sternutatory reflex, solar sneeze reflex, and the acronym "ACHOO" (autosomal dominant compelling hello-ophthalmic outburst syn­drome) have been applied to describe what has been characterized as an uncontrollable sneeze in response to light stimulation or the inherited trait of sneezing in response to light stimulation. The term "reflex" should be rejected as a component of the response of sneezing to light stimulation because the sneeze is only variably triggered by such stimulation. Other light sources have been demonstrated to pro­duce a sneeze, so the narrow "solar sneeze" would not apply universally. ACHOO fails to allow for any

potential for an acquired mechanism explaining the sneezing response among those patients without a distinct familial pattern. The term "photic sneeze response" is preferable because it is clinically descriptive and unencumbered by the equivocal assumption of an autosomal dominant inheritance pattern. The majority (87.8%) of the PSR subjects in the present study recognized their photic sneeze response before the age of 30 years. While only 13.6% (9 of 66 respondents) of PSR patients in the study indicated that photic sneezing began before the age of 10 years, previous studies suggest the autoso­mal dominant mode of inheritance. ~>9 Additionally, the response with a sneeze or series of sneezes appears from these findings to have enough variability to shrink from being described as a reflex. The incon­sistency of familial presence of the PSR opens the possibility of an acquired mechanism and is support­ed by the current findings.

Among the study participants, statistically significant correlations were found between photic sneeze response and white race (115/367; 38.2%, p=0.0001) and presence of deviated nasal septum (9116 respondents who were aware of the presence or absence of the condition; p=0.0007). From the data compiled in the present study; photic sneezing appears to be recognized early in life. Most of the respondents (87.8%) had recognized the phenome­non by the age of 30 years; over half of them (53.0%) by the age of 15 years. This response was obtained even though only 23.2% of photic sneezers had one parent who exhibited the response. Lack of recogni­tion of the sneeze response is not intended to imply lack of distinct genetic pattern of inheritance.

Consistent with previous reports on the prevalence of autosomal dominant inheritance, 21 of 79 PSR re­spondents (26.5%) indicated an awareness of one or both parents exhibiting a photic sneeze response. In response to the question concerning relatives exhibit­ing the PSR, 48n9 of sneezers (60.8%) failed to indi­cate the presence of the response in one or both par­ents. Reports focusing on the inheritance pattern of PSR suggest that a majority of subjects with PSR inherit the trait.6.8 Study data suggest, based on responses to the survey, that in approximately 25% of cases the PSR may be inherited in an autosomal dom­inant manner, but that the majority of cases appear to be related to environmental influences. If the cases indicating an inheritance pattern are extrapolated for prevalence from the data of Beckman and Nordenson', 18/110 (16.4%) pedigrees demonstrated AD inheri­tance. The preponderance of population-based stud-

375

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.JOURNAL OF THE AMERICAN OPTOMETRIC ASSOCIAT I ON VOLUME 68/HUMBER 6/JUNE 95

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(64.7%) , and yawning (14.4%).

With regard to evidence for a genetic transmittance pattern of PSR, 48 of 122 PSR respondents (39.3%)

Average number of seconds between successive sneezes

caused by sunlight, among sneezers

*39 repeat sneezers did not respond

Table 4 reported that no direct relatives were also photic sneezers. In fact , only 21 of 79 photic sneezers (1 7.2%) indicated that either parent experienced the photic sneeze response.

Other stimuli to sneezing identified in the question­naire included automobile heacWghts, lamps in doc­tors' offices, <Uld pollen. Eighty-six of 122 sneezers (70.5%) reported that pollen was a stimulus to sneez­ing. Thirty-one of 122 (25.4%) reported that head­lights or floodlights also prompted a sneezing response.

Discussion Sneezing in response to light has various names. Terms such as the photo-sternutatory reflex, solar sneeze reflex, and the acronym "ACHOO" (autosomal domimmt compelling hello-ophthalmic outburst syn­drome) have been applied to describe what has been characterized as an uncontrollable sneeze in response to light stimulation or the inherited trait of sneezing in response to light stimulation. The term "reflex" should be rejected as a component of the response of sneezing to light stimulation because the sneeze is only variably triggered by such stimulation. Other light sources have been demonstrated to pro­duce a sneeze, so the narrow "solar sneeze" would not apply universally. ACHOO fails to allow for any

potential for an acquired mechanism explaining the sneezing response among those patients without a distinct familial pattern. The term "photic sneeze response" is preferable because it is clinically descriptive and unencumbered by the equivocal assumption of an autosomal dominant inheritance pattern. The majority (87.8%) of the PSR subjects in the present study recognized their photic sneeze response before the age of 30 years. While only 13.6% (9 of 66 respondents) of PSR patients in the study indicated that photic sneezing began before the age of 10 years, previous studies suggest the autoso­mal dominant mode of inheritance. 6·9 Additionally, the response with a sneeze or series of sneezes appears from these findings to have enough variability to shrink from being described as a reflex. The incon­sistency of familial presence of the PSR opens the possibility of an acquired mechanism and is support­ed by the current findings.

Among the study participants, statistically significant correlations were found between photic sneeze response and white race (115/367; 38.2%, p=O.OOOI) and presence of deviated nasal septum (9116 respondents who were aware of the presence or absence of the condition; p=0.0007). From the data compiled in the present study, photic sneezing appem·s to be recognized early in life. Most of the respondents (87.8%) had recognized the phenome­non by the age of 30 years; over half of them (53.0%) by the age of 15 years. This response was obtained even though only 23.2% of photic sneezers had one parent who exhibited the response. Lack of recogni­tion of the sneeze response is not intended to imply lack of distinct genetic pattern of inheritance.

Consistent with previous reports on the prevalence of autosomal dominant inheritm1ce, 21 of 79 PSR re­spondents (26.5%) indicated m1 awareness of one or both parents exhibiting a photic sneeze response. In response to the question concerning relatives exhibit­ing the PSR, 48179 of sneezers (60.8%) failed to indi ­cate the presence of the response in one or both par­ents. Reports focusing on the inheritance pattern of PSR suggest that a majority of subjects with PSR inherit the trait.6

·8 Study data suggest, based on

responses to the survey, that in approximately 25% of cases the PSR may be inherited in an autosomal dom­inmlt manner, but that the majority of cases appear to be related to environmental influences. If the cases indicating an inheritance pattern are extrapolated for prevalence from the data of Beckman and Nordenson' , 18/110 (16.4%) pedigrees demonstrated AD inheri­tance. The preponderance of population-based stud-

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JOURNAL OF THE AMERICAN OPTOMETRIC ASSOCIATION VOLUME 66/N UM BER 6/JUNE 95

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ies seem to suggest that only a minority of cases exhibiting the PSR are inherited.

For example, the strong association between photic sneezing and deviated nasal septum, and the weaker association with tobacco use, support the likelihood of an acquired mechanism. The numbers of these respondees, however, were too small for meaningful statistical analysis. A positive relationship could not be found between the systemic disorders queried and photic sneezing. The probability of mixed or separate causality mechanisms (inherited or acquired) for the PSR, therefore, should be entertained.

Of the PSR population, only 15 of 122 (12.3%) admit­ted to sneezing almost every time they were exposed to sunlight, while the majority (63/122; 51.6%) admitted to sneezing only occasionally in response to sunlight. Of 118 sneezers reporting frequency, most (107; 90.1 %) responded with between 1 and 3 sneezes caused by sunlight. The majority (85.0%) of the PSR population reported a latency of between 0 and 19 seconds between successive sneezes (Table 4) . This self-report is in interesting concert with the only report of a laboratory measurement of latency, which was 9.9 sec (range 8.6 to 12 sec).'9 Seasonal inlluence on photic sneezing was absent in 50.0% of cases.

From previous studies it has been shown that the PSR is present in approximately 30% of selected popula­tions in the USA, Sweden, and Great Britain.5•7•9•10 The current data, which possibly represent the second largest population survey conducted to date, indicate a prevalence of 33.2% (1221367), which is compara­ble to that reported from Swedish blood donors (24%).7 The current prevalence data are also consis­tent with Everett's reported prevalence of 33.3% among psychiatry house officers.5 His selected popu­lation, however, included only 18 subjects. The presently reported data encompasses 367 subjects. A summary of previously reported studies on the phot­ic sneeze response is presented in Table 5; on average these previous studies have found the PSR in 26.5% of patients.

The photic sneeze response has been reported to fol­low a pattern of autosomal dominant inheritance among studies reporting this statistic.6

·" Beckman and Nordensen\ however, characterized the photic sneeze response as a "threshold trait", in which varying stim­uli are potential triggers for the response. Descriptive factors among a general clinic population included the following:

• sunlight as the stimulus (6/6; 100%) • onset at birth or earliest remembrance

(3/6; 50%)

TABLE 5 .................................................................... ................. .......................................................... ..... ............ ....................................................................................

JOURNAL OF THE AMERICAN OPTOMETRIC ASSOCIATION VOLUME 66/NUMBER 6 I JUNE 95

PR I MARY EYECARE CLINIC

ies seem to suggest that only a minority of cases exhibi ting the PSR are inherited.

For example, the strong association between photic sneezing and deviated nasal septum, and the weaker association with tobacco use, support the likelihood of an acquired mechanism. The numbers of these respondees, however, were too small for meaningful statistical analysis. A positive relationship could not be found between the systemic disorders queried and photic sneezing. The probability of ll1Lxed or separate causality mechanisms (inherited or acquired) for the PSR, therefore, should be entertained.

Of the PSR population, only 15 of 122 (12.3%) admit­ted to sneezing almost eveq time they were exposed to sunlight, while the majority (631122; 51.6%) admitted to sneezing only occasionally in response to sunlight. Of 118 sneezers reporting frequency, most (I 07; 90.1 %) responded with between l and 3 sneezes caused by sunlight. The majority (85.0%) of the PSR population reported a latency of between 0 and 19 seconds between successive sneezes (Table 4) . This self-report is in interesting concert with the only report of a laborat01y measurement of latency, which was 9.9 sec (range 8.6 to 12 sec).'" Seasonal influence on photic sneezing was absent in 50.0% of cases.

From previous studies it has been shown that the PSR is present in approximately 30% of selected popula­tions in the USA, Sweden, and Great Britain. 1

·7·" '

0 The current data, which possibly represent the second largest population survey conducted to date, indicate a prevalence of 33.2% (1221367), which is compara­ble to that reported from Swedish blood donors (24%) _~ The current prevalence data are also consis­tent with Everett 's reported prevalence of 33.3% among psychiatly house officers.1 His selected popu­lation , however, included only 18 subj ects. The presently reported data encompasses 367 subjects. A sumnmy of previously reported studies on the phot­ic sneeze response is presented in Table 5; on average these previous studies have found the PSR in 26.5% of patients.

The photic sneeze response has been reported to fol ­low a pattern of autosomal dominant inheritance among studies reporting this statistic."' Beckman and Nordensen7

, however, characterized the photic sneeze response as a "threshold trait", in which va1yi ng stim­uli are potential triggers for the response. Descriptive factors among a general clinic population included the following:

• sunlight as tl1e stimulus (6/6; 100%) • onset at birth or earliest remembrance

(316; 50%)

Comparison of prevalence of PSR in previous surveys Author (s}/year Study population Prevalence

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JOURNAL OF THE AM E RICAN OP T OMETRIC A S SOCIATION VOLUME 66/ NUMBER 6 / JUNE 95

................................ ............ .. ........................ .. .. ........ .. .......... ...... .. ...... ...... ........ .... ........ .. .................................................. ~.~ .. ·. !~'! .~ .. ~.! ... ~.~-~-~-~-~- ~- - -~ .. 1:-. .' .. ~.! .~.

• absence of history of allergy ( 6/6; I 00%) • nasal disorders (5/6; 83%) [3, chronic

partial nasal obstruction, I deviated septum,' sinusitis].'8

In their recent literature review, Whitman and Packer'9 contend that an unrecognized threshold response may allow those exhibiting the response to the stimulus of light to complete a frustrated or sus­pended sneeze.

Whether the photic sneeze response is consistent with a pattern of autosomal dominant inheritance or con­sistently associated with environmental factors such as deviated nasal septum or tobacco use, remains unanswered. The current data present the possibility of an acquired mechanism or genetic inheritance to explain the presence of the PSR.

Summary Sneezing in response to bright light stimuli is readily recognized in those who have experienced the phe­nomenon and may be met with astonishment by those who are unfamiliar with it. It is a phenomenon occa­sionally enquired about by patients, who may notice its association with exposure to bright lights, espe­cially sunlight. PSR is in general a benign condition.

The PSR may prove to be an annoyance, especially among patients who perform tasks requiring concen­tration such as pilots, drivers, photographers, sur­geons, and those involved in outdoor athletics. Practitioners should be prepared to discuss this response and caution these patients concerning movement from bright to dim environs. 20 Although avoidance of bright light may be the only known solu­tion, recognition that the phenomenon is likely to occur in certain situations may prepare the patient for its occurrence, and reassure the patient that PSR in and of itself is not a dangerous condition.

References 1. Watson WS. Diseases of the nose and its accessory cavities . London: Lewis, 1875; 343·4.

2. Murray N. Bierer J. Prolonged sneezing. A case report. Psychosom Med 1951 ; 13:56·8.

3. Shilkret HH. Psychogenic sneezing and yawning. Psychosom Med 1959; 11 :127·8.

4. Kolman 0. Paroxysmal sneezing. Can Med Assoc J 1964; 91:154·7.

5. Everett HC. Sneezing in response to light. Neurology 1964; 14:483·90.

6. Collie WR, Pagon RA. Hall JG, et al. ACHOO syndrome (autosomal dominant compelling helio-ophthalmic outburst syndrome). Birth Defects: Original Article Series 1978; 14 (6B); 361-3.

7- Beckman L, Nordenson I. Individual differences with respect to the sneezing reflex: An inherited physiological trait in man7 Hum Hered 1983; 33(6):390-1 .

8. Peroutka SJ, Peroutka LA. Autosomal dominant transmission of the "photic sneeze reflex" (letter). N Eng J Med 1984; 310(9):599-600.

9. Forrester JM. Sneezing on exposure to bright light as a inherited response. Hum Hered 1985; 35(2):113-4.

10. Lang OM. Howland WC. Solar sneeze reflex. JAMA 1987; 257(10): 1330-1.

11 . Sedan J. Photosternutatory reflex. Rev Otoneuroophthalmol 1954; 26:123-6.

12. Birch CA. Sneezing. Practitioner 1959; 182:122-4.

13. Lewkonia I. An infrequent response to slit-lamp examination. Br J Ophthalmol 1969; 53(7):493-5.

14. Askenasy JJM. The photic sneeze. Postgrad Med J 1990; 66(781 ):892-3.

15. Pies R. Seasonal affective disorder and the photic sneeze response. Am J Psychiatry 1990; 147(8): 1094.

1 6. Smith R. Photic sneezes (editorial). Br J Ophthalmol 1990; 74(12):705.

17. Katz B, Melles RB. Swenson MR. et al. Photic sneeze reflex in nephropathic cystinosis. Br J Ophthalmol1990; 74(12):706-8.

18. Morris HH. ACHOO syndrome: Laboratory findings. Cleve Clin J Med 1989; 56(7):743-4.

19. Whitman BW. Packer RJ . The photic sneeze reflex: Literature review and discussion. Neurology 1993; 43(5):868-71.

20. Benbow EW. Practical hazards of photic sneezing. Br J Ophthalmol 1991; 75(7):447.

Submitted 11/94; Accepted 5/95 Leo P. Sames, 0 . 0 .

School of Optometry University of Alabama at Birmingham

Birmingham, AL 35294

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