Role of intrauterine Rubella infection in the causation of congenital deafness.

Background : Rubella is a contagious viral infection associated with rash. 10-15% of women are susceptible to this infection in their childbearing years and the disease is usually transmitted to the developing fetus causing abortion or stillbirth or congenital Rubella syndrome (CRS). Sensorineural hearing loss is one of the most common abnormalities associated with CRS. Aims : The study was undertaken to determine the prevalence of Rubella induced congenital deafness in south India. Materials and Methods :The present study is carried out on 1076 hearing impaired children below 14 years of age to determine etiological factors for congenital hearing impairment. Various audiometric tests such as PTA, OAE and BERA were carried out to know the type and degree of hearing impairment. Development histories including the prenatal, perinatal and postnatal histories were collected using a standard questionnaire. Results :The results indicated very less percentage (1.57%) of cases with the history of maternal Rubella infection indicating a significant reduction of Rubella-induced deafness in the developing countries. Conclusions : The main reason behind the low percentage of children with rubella- induced hearing impairment may be due to appropriate immunization of mothers to the rubella infection during their child bearing years. The increasing awareness in the developing countries of this infection and its consequences is another important reason behind the low percentage of the affected population.

Immunological studies in children with hearing impairment.

An immunological study was carried out on 58 children below 14 years of age with sensorineural hearing loss of unknown aetiology. The observed elevated levels of IgE in 25.86% (n = 15) children and antinuclear antibodies in 10.34% (n = 6) children indicate that auto-immune activity has a role in the causation of hearing impairment.

Facio-auricular vertebral syndrome-a case report.

Facio Auricular Vertebral (FAV) or Goldenhar syndrome is a very rare kind of syndromic deafness and is inherited as autosomal dominant. A study was taken up to understand the prevalence of this syndrome in children below the age of 14 years with hearing loss. Out of 1073 children with hearing impairment, Goldenhar syndrome was observed only in 1 (0.09%) case. The child suffered severe hearing loss. Facial paralysis and hemifacial microsomia were prominent features observed in the child. Facio-Auricular-Vertebral syndrome is therefore synonymously used with Goldenhar syndrome.