Journal of Otology & RhinologyISSN: 2324-8785

All submissions of the EM system will be redirected to Online Manuscript Submission System. Authors are requested to submit articles directly to Online Manuscript Submission System of respective journal.
bahis siteleri bahis siteleri bahis siteleri casino siteleri

Case Report, J Otol Rhinol Vol: 4 Issue: 5

Case Series of all three types of Ushers Syndrome of varying severity in the same family

Neha Thakur* and Narendra Rai
Department of Pediatrics, Hind Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
Corresponding author : Dr. Neha Thakur MD
Assistant Professor, Hind Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
Tel: +917607804653
Received: August 20, 2015 Accepted: September 17, 2015 Published: September 21, 2015
Citation: Thakur N, Rai N (2015) Case Series of all three types of Ushers Syndrome of varying severity in the same family. J Otol Rhinol 4:5. doi:10.4172/2324-8785.1000251


Introduction: Ushers syndrome is an autosomal recessive disorder characterized by hearing loss and visual impairment due to retinitis pigmentosa. There are three types of Ushers syndrome with decreasing order of severity. Type 1 has onset of illness at birth with rapid progression of symptoms. Type 2 has gradual onset of symptoms with moderate hearing loss and later onset of night vision problems. Type 3 is the least severe form with onset of symptoms at late teenage.

Case Series: We report here four siblings with Ushers. The first and second child had clinical symptoms suggestive of type1 Ushers. Third child has clinical features of Type 3 Ushers. Fourth child who had presented to us was having clinical features supportive of type 2 Ushers. There was a family history of similar symptoms in their cousin brother. All the four siblings were product of consanguineous marriage. Their mother was suffering from discoid lupus erythematosus.

Conclusion: Timely diagnosis of Ushers could have decreased the morbidity of these children. Universal Screening for newborn hearing at birth will help in early detection of children with hearing loss and associated syndromes. Cochlear implantation is the only treatment in Ushers and earlier the implantation better is the prognosis. Hence in developing countries like India where genetic diagnosis is not possible most of the times due to financial constraints we can at least implement programs like hearing screening at birth.

Keywords: Night blindness; Hearing loss; Ushers Syndrome

international publisher, scitechnol, subscription journals, subscription, international, publisher, science

Track Your Manuscript

Awards Nomination

Recommended Conferences

Media Partners


open access