None of the most common types of deletions in the GJB2 gene (c.35delG, c.167delT or c.235delC) were found in this large cohort of deaf children from Uganda. This prompts a search for genetic causes of deafness among this and other previously studied African populations.

BackgroundThe Uganda Hearing Project is a non-profit program assisting with teaching of ear surgery in Uganda. The project started with cadaveric temporal bone courses in 2003 and 2005, including donation of operating microscopes and ear instruments. In 2006, three surgical groups started regular surgical teaching visits.MethodsA retrospective chart review of all cases of middle ear surgery performed in Uganda from 2003 to 2009. Surgeries by local surgeons without foreign presence were coded as ‘local’ and those performed with assistance of visiting surgeons were coded as ‘visitors’.ResultsIn 2005, two middle ear surgeries using the operating microscope were done in the Ugandan teaching hospitals by Ugandan Otolaryngologists alone. From the onset of surgical visits in 2006, a total of 193 middle ear surgeries were performed - 115 tympanomastoidectomies, 77 tympanoplasties, and 1 cochlear implant. In 2006 (one surgical teaching visit), 6 middle ear surgeries were performed with visiting surgeon presence and 2 surgeries were performed by the local team alone. This increased in 2007 (2 visits) and again in 2008 (3 visits) to 34 cases with visiting surgeon presence and 48 local cases.ConclusionsThe temporal bone courses and donation of operating microscopes to Ugandan hospitals have revolutionized middle ear surgery in Uganda. The surgical visits by the Uganda Hearing Project have led to a 24-fold increase in annual middle ear surgeries performed with the operating microscope by Ugandan Otolaryngologists. Increased frequency of surgical visits was correlated with an increase in local surgical output, hopefully resulting in improved care for Ugandans with ear disorders.

Hearing impairment is a significant disability. According to the World Health Organization (WHO), more than 80% of the world's approximately 120 million people with hearing impairment live in developing countries. Cochlear implant is the only therapeutic intervention for those with severe-profound sensorineural hearing loss. We are reporting an interesting case of the very first cochlear implant operation carried out in Uganda. The patient was a 23 year old male whose presenting complaint was inability to hear in the left ear for three and a half years and in the right ear for one year. He had been treated for TB(Tuberculosis) mastoiditis . After the 8 months of treatment, the otorrhea persisted and he underwent a tympanomastoidectomy on the same ear. He reported no familial history of hearing loss. On examination, ENT examination revealed a small pars flaccida retration pocket of the right tympanic membrane with cholesteatoma. The left ear had an intact tympanic membrane. Pure tone audiometry revealed profound sensorineural hearing loss in both ears( see attached PTA results), CT scan of the temporal bone showed normal inner ear anatomy bilaterally and mild sclerotic changes in both mastoid bones. He then had surgery on his right ear which included cochlear implantation. The cochlear implant (CI) was activated on the first postoperative day remotely via internet with the help of the cochlear implant team at New York University Cochlear Implant Center and the patient was immediately able to appreciate some sounds. He received a pneumococcal vaccine on the first postoperative day and was discharged the following day.