6533b829fe1ef96bd128ad3f
RESEARCH PRODUCT
Hearing loss in facioscapulohumeral dystrophy.
T. VoitAntoinette LamprechtH. G. LenardHans H. Goebelsubject
musculoskeletal diseasesmedicine.medical_specialtyShoulderAdolescentHearing lossBiopsyFacial MusclesAudiologyMuscular DystrophiesBasal (phylogenetics)Atrophyotorhinolaryngologic diseasesMedicineHumansMuscular dystrophyChildHearing LossHearing Loss High-FrequencyCochleaEarly onsetRetrospective Studiesbusiness.industryHearing TestsMusclesDystrophySyndromemedicine.diseaseMuscular AtrophyPediatrics Perinatology and Child HealthSensorineural hearing lossmedicine.symptombusinessdescription
Bilateral sloping high frequency hearing loss of 20–90 dB was found in six out of ten patients with infantile or adolescent onset FSHD. In all cases the basic defect could be traced to the cochlea. The outer hair cells of the basal turn are predominantly affected. In 20 patients with various other forms of muscular dystrophy or neuromuscular disorders with an FSH distribution, no sensorineural hearing loss was found. Myopathology of FSHD patients extended from mild to severe, often showing inflammatory infiltrates and type I fibre atrophy, without unequivocal differences between the two groups with and without hearing loss. It is concluded that cochlear dysfunction is a specific and frequenct phenomenon of early onset FSHD.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 1986-09-01 | European journal of pediatrics |