title: Hereditary hearing loss--the role of environmental factors.
abstract: A large family with mid onset sensorineural hearing loss (HL) was used to study the effect of environmental factors on progression HL. Data from five-generations of one family were traced and 104 living members were included in the study. Audiograms were measured for 60 family members. We used an expert program to study the individual risk factors, which included the known risk factors for sensorineural HL. The pattern of inheritance in this HL family was autosomal dominant and 22 individuals were affected. The HL among affected individuals was symmetrical and varied from mild to severe. The mean age at onset of HL was 22 years. The mean deterioration of hearing at 2 kHz frequency was 1 dB/year. In the risk analysis the use of NSAID-analgetics and military noise exposure correlated with HL. There was no correlation between occupational and free time noise-exposure and HL. Analgetics may aggravate the HL in subjects with inherited HL. This type of genetic lesion is new and is not in those chromosomal areas already documented in the literature.
authors.affiliation: Department of Otolaryngology, Karolinska Institute, Stockholm, Sweden.
authors.forename: R
authors.initials: R
authors.lastname: Kaksonen
authors.forename: I
authors.initials: I
authors.lastname: Pyykkö
authors.forename: J
authors.initials: J
authors.lastname: Kere
authors.forename: J
authors.initials: J
authors.lastname: Starck
authors.forename: E
authors.initials: E
authors.lastname: Toppila
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000483:Alleles; D001299:Audiometry / Q000379:methods; D002648:Child; D004781:Environmental Exposure / Q000009:adverse effects*; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology* / Q000235:genetics*; D006801:Humans; D008875:Middle Aged; D017354:Point Mutation / Q000235:genetics; D012307:Risk Factors; D012720:Severity of Illness Index