title: Neurological associations in auditory neuropathy spectrum disorder: Results from a tertiary hospital in South India.
abstract: OBJECTIVE
  To find out the prevalence and types of neurological abnormalities associated in auditory neuropathy spectrum disorder in a large tertiary referral center.
  
  
  METHODS
  A prospective clinical study was conducted on all patients diagnosed with auditory neuropathy spectrum disorder in the ear, nose, and throat (ENT) and neurology departments during a 17-month period. Patients with neurological abnormalities on history and examination were further assessed by a neurologist to determine the type of disorder present.
  
  
  RESULTS
  The frequency of auditory neuropathy spectrum disorder was 1.12%. Sixty percent were found to have neurological involvement. This included cerebral palsy in children, peripheral neuropathy (PN), spinocerebellar ataxia, hereditary motor-sensory neuropathy, spastic paresis, and ponto-bulbar palsy. Neurological lesions did not present simultaneously with hearing loss in most patients. Sixty-six percent of patients with auditory neuropathy spectrum disorder were born of consanguineous marriages.
  
  
  CONCLUSIONS
  There is a high prevalence of neurological lesions in auditory neuropathy spectrum disorder which has to be kept in mind while evaluating such patients. Follow-up and counselling regarding the appearance of neuropathies is therefore important in such patients. A hereditary etiology is indicated in a majority of cases of auditory neuropathy spectrum disorder.
authors.affiliation: Department of Ear, Nose and Throat, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
authors.forename: Anjali
authors.initials: A
authors.lastname: Lepcha
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, Hamad Medical Corporation, Al Wakra Hospital, Doha, Qatar.
authors.forename: Reni K
authors.initials: RK
authors.lastname: Chandran
authors.affiliation: Department of Neurology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
authors.forename: Mathew
authors.initials: M
authors.lastname: Alexander
authors.affiliation: Department of Ear, Nose and Throat, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
authors.forename: Ann Mary
authors.initials: AM
authors.lastname: Agustine
authors.affiliation: Department of Ear, Nose and Throat, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
authors.forename: K
authors.initials: K
authors.lastname: Thenmozhi
authors.affiliation: Department of Ear, Nose and Throat, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
authors.forename: Achamma
authors.initials: A
authors.lastname: Balraj
keywords: Auditory neuropathy; Charcot- marie-tooth disease; Friedreich ataxia; Refsum disease; consanguinity; genetic counseling; hereditary sensory; motor neuropathy; neurologic dysfunction; sensorineural hearing loss