Shejal Kasera¹*, Sachidanand Sinha² and Mangal Chandra Yadav³

¹Department of ENT, Mahatma Gandhi Memorial Medical College, Indore, M.P., India
²Department of ENT, Pandit Jawahar Lal Nehru Memorial Medical College, Raipur, Chhattisgarh, India
³Department of ENT, All India Institute of Medical Sciences, Rishikesh, Uttrakhand, India

*Corresponding Author: Shejal Kasera, Department of ENT, Mahatma Gandhi Memorial Medical College, Indore, M.P., India.

Received: April 11, 2022; Published: May 23, 2022

Abstract

Kallmann Syndrome (KS) was characterized by hypogonadotropic, hypogonadism (HH) and anosmia/hyposmia with developmental anomalies of the olfactory bulb and hypothalamus. It has a heterogeneous inheritance pattern that can be x-linked autosomal dominant or autosomal recessive disorder. There are currently more than 20 pathogenic genes linked to KS, six of which are quite frequent (KAL1, FGFR1, PROKR2, PROK2, CHD7, FGF8). KS affects approximately 1 in every 10,000 males and 1 in every 40,000 females. Micropenis and cryptorchidism are common in males with KS, as well as the development of secondary sexual characteristics such as deepening of voice and growth of facial hair, while menstruation and breast development are noted in females. Other developmental deficits are also associated with KS, such as hearing impairment, eye related issues, cleft lip or palate, atypical tooth development.

In this study, we have described two case reports of KS, who had hearing loss. Detailed audiological and ENT evaluation were carried out including otoscopic examination, Immittance evaluation, Puretone audiometry, Otoacoustic emissions, Auditory Brainstem implant and Hearing aid trial. Radiological evaluation was lao done and the results were in correspondence with previously published literature. The cruras and footplate of the stapes, as well as the oval window, were absent. The stapes head and neck were made up of a little piece of bone that was linked to the incus' lenticular process. The stapedius tendon was present and connected to the stapes' neck. The facial nerve was lying at a lower position than expected in its tympanic portion.

The implications of the test findings suggest that the early diagnosis of KS is based on multidisciplinary cooperation are important for the treatment process. Puberty development is also important for skeletal, metabolic, and psychological consequences, in addition to sexual health. If KS is detected early, hormone replacement can be started, and secondary sexual development can resume. Adequate genetic counseling can also be provided to family members.

Keywords: Kallmamm Syndrome; Hypogonadotropic Hypogonadism; Conductive Hearing Loss

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Citation

Citation: Shejal Kasera., et al. “Systematic Audiological Assessment of Individuals with Kallmann Syndrome: In the Indian Population".Acta Scientific Otolaryngology 4.6 (2022): 62-66.

Copyright

Copyright: © 2022 Shejal Kasera., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.