title: Auditory function in women with autoimmune inner ear diseases and their offspring.
abstract: OBJECTIVE
  The precise cause of congenital sensorineural hearing loss (CSNHL) is unclear in many cases. In a previous study we found that offspring from guinea pigs with autoimmune sensorineural hearing loss (ASNHL) exhibited signs of SNHL. Here we studied women with autoimmune inner ear diseases (AIED) and their offspring. Our aim was to determine if autoimmune damage may be one of the causes of CSNHL.
  
  
  METHODS
  Thirty-eight pregnant women with AIED were recruited. Thirty-three had ASNHL; one with autoimmune delayed endolymphatic hydrops (ADEH) and four with autoimmune Meniere's disease (AIMD). The following were assessed in all women: audiogram, auditory brain stem response (ABR), otoacoustic emission (OAE), vestibular function test and presence of inner ear antigens. The following were assessed in offspring from these women: OAE, ABR and presence of inner ear antigens.
  
  
  RESULTS
  Five of the 38 children born to women with AIED had SNHL (an incidence much higher than normal). OAEs were not inducible in these children shortly after birth or within 46-100 days after birth. Abnormal ABR findings were apparent in these five children and inner ear antigens were detected in three of the five children (the mother's of these children were also positive for inner ear antigens).
  
  
  CONCLUSIONS
  These preliminary findings suggest that the prevalence of congenital ASNHL may be increased in offspring born to women with AIED.
authors.affiliation: Department of Otolaryngology, and Otolaryngological Science Research Unit, First Affiliated Hospital of Nanjing Medical University, 300 Guangzhou Road, Nanjing 210029, Jiangsu Province, China.
authors.forename: Chang-qiang
authors.initials: CQ
authors.lastname: Tan
authors.forename: Wei-da
authors.initials: WD
authors.lastname: Dong
authors.forename: Lang
authors.initials: L
authors.lastname: Guo
authors.forename: He
authors.initials: H
authors.lastname: Huang
authors.forename: Deng-yuan
authors.initials: DY
authors.lastname: Wang
mesh_terms: D000328:Adult; D001301:Audiometry, Pure-Tone; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000453:epidemiology; D016022:Case-Control Studies; D002675:Child, Preschool; D015331:Cohort Studies; D016057:Evoked Potentials, Auditory, Brain Stem; D005260:Female; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000151:congenital* / Q000175:diagnosis* / Q000453:epidemiology; D006320:Hearing Tests; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007759:Labyrinth Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000276:immunology; D017084:Otoacoustic Emissions, Spontaneous; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology*; D012016:Reference Values; D018570:Risk Assessment; D014724:Vestibular Function Tests; D055815:Young Adult