There are a large number of well recognised syndromes comprising cerebellar ataxia in association with other neurological features. We report three family members who presented with a relapsing, early onset cerebellar ataxia, associated with progressive optic atrophy and sensorineural deafness. All three patients have areflexia (in the absence of a peripheral neuropathy), a pes cavus deformity,...

Sudden sensorineural hearing loss is defined as sudden, idiopathic, usually unilateral deafness developed at most within 72 hours in previously healthy person. This paper presents very rarely occurring case of sudden sensorineural hearing loss in 23-year-old healthy nulliparous women during an uncomplicated pregnancy. The possibility of therapy of that sudden deafness in pregnant women is restr...

Although etiological studies have shown genetic disorders to be a common cause of congenital/early-onset sensorineural hearing loss, there have been no detailed multicenter studies based on genetic testing. In the present report, 264 Japanese patients with bilateral sensorineural hearing loss from 33 ENT departments nationwide participated. For these patients, we first applied the Invader assay...

Aim: To identify the role of consanguinity in the appearance of deafness in children of consanguineously married couples. Materials & methods: This retrospective, record based, survey type study was carried out during April-July, 2007. We completed a questionnaire for each student after interviewing the student and the parent or guardian. We also consulted the record at the school. The data was...

Sixty children who survived meningitis during the outbreak of meningococcal group B infection in Bolton 1971-74 were assessed between 5 and 9 years later. Each case, together with a matched control, has been examined clinically and subjected to a number of psychological tests and to routine audiology. The results, unlike those from other series, did not demonstrate any incoordination, ataxia, o...

Alport syndrome (ATS) is a type-IV collagen inherited disorder, caused by mutations in COL4A3 and COL4A4 (autosomal recessive) or COL4A5 (X-linked). Clinical symptoms include progressive renal disease, eye abnormalities and high-tone sensorineural deafness. A renal histology very similar to ATS is observed in a subset of patients affected by mutations in MYH9, encoding non-muscle-myosin Type II...

Bone Conduction Hearing aids are used mainly for the treatment of patients who suffer from Single Sided deafness and/or Conductive/ mixed hearing loss but not Bilateral Sensorineural loss. Vibration Acoustic Stimulation is a combination both air and bone conduction at same time, four aids, two ears bilateral one aid, on defective ear single sided (SSD) Unilateral Loss. We have tried this approa...

autoimmune disorders, most notably myasthenia gravis (MG) which is seen in up to 40% of cases.1,2 There are also a number of other less common autoimmune disorders associated with thymoma, including hyperexcitability syndromes,3 myositis,1 encephalitis,4 autonomic dysfunction,5 and lupus.6 In 2004 Vernino and Lennon7 reported autoimmune sensorineural hearing loss (SNHL) in two patients from the...