scholarly journals A rare case of Waardenburg syndrome with unilateral hearing loss caused by nonsense variant c.772C>T (p.Arg259*) in the MITF gene in Yakut patient from the Eastern Siberia (Sakha Republic, Russia)

2019 ◽  
Vol 78 (1) ◽  
pp. 1630219 ◽  
Author(s):  
Nikolay A. Barashkov ◽  
Georgii P. Romanov ◽  
Uigulaana P. Borisova ◽  
Aisen V. Solovyev ◽  
Vera G. Pshennikova ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Rare Case ◽  
Unilateral Hearing Loss ◽  
Waardenburg Syndrome ◽  
Eastern Siberia ◽  
Sakha Republic

Unilateral hearing loss as primary symptom of craniopharyngioma in a six-year-old girl

2008 ◽  
Vol 122 (3) ◽  
Author(s):  
R Hofman ◽  
H J Rosingh
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Sensorineural Hearing Loss ◽  
Otoacoustic Emissions ◽  
World Literature ◽  
Rare Case ◽  
Sensorineural Hearing ◽  
Unilateral Hearing Loss ◽  
Magnetic Resonance Scanning ◽  
Internal Acoustic Canal

AbstractObjective:We report a rare case of otological presentation of craniopharyngioma.Method:Case report and review of world literature concerning presentations of craniopharyngioma.Results:A six-year-old girl was referred to our department with unilateral hearing loss. This appeared to be a complete sensorineural hearing loss. Otoacoustic emissions were reproducible on both sides. Magnetic resonance scanning revealed a massive, cystic craniopharyngioma exerting pressure on the patient's ventricular system and brainstem and also invading the internal acoustic canal. The mass was resected via a craniotomy. The patient's hearing loss completely recovered, and she experienced no neurological or endocrinological side effects of the treatment. Craniopharyngioma have a prevalence of 0.13–2:100 000.Conclusion:Craniopharyngioma is a rare disease. First presentation with otological symptoms is extremely rare. Otoacoustic emissions can differentiate between cochlear and retrocochlear causes of sensorineural hearing loss.

scholarly journals A Rare Case of a Sebaceous Nevus in the External Auditory Canal

2003 ◽  
Vol 82 (1) ◽  
pp. 38-41 ◽  
Author(s):  
Nici Eddy Bothwell ◽  
Craig C. Willard ◽  
Douglas M. Sorensen ◽  
Timothy J. Downey
Keyword(s):  
Hearing Loss ◽  
Differential Diagnosis ◽  
External Auditory Canal ◽  
Rare Case ◽  
Unilateral Hearing Loss ◽  
Essential Information ◽  
Secondary Neoplasm ◽  
The Face ◽  
Three Stages ◽  
Different Characteristics

We report the case of a patient who came to us for evaluation of a progressive unilateral hearing loss and who was found to have a sebaceous nevus in an unusual location: the external auditory canal. A sebaceous nevus is a congenital organoid mass that occurs primarily on the face, scalp, and periauricular regions. Despite the predilection of sebaceous nevi for the head and neck, reports of this lesion rarely appear in the otolaryngology literature. Left untreated, the lesion can progress through three stages of gross and histopathologic development; a sebaceous nevus begins as a small benign papule, grows into an enlarging mass with different characteristics, and ultimately becomes a secondary neoplasm. The lesion's potential for malignant transformation and its association with syndromes underscores the importance of prompt recognition and appropriate management. This case report adds the sebaceous nevus to the differential diagnosis of external auditory canal lesions and provides essential information about this rare mass.

scholarly journals Allelic Mutations of KITLG, Encoding KIT Ligand, Cause Asymmetric and Unilateral Hearing Loss and Waardenburg Syndrome Type 2

2015 ◽  
Vol 97 (5) ◽  
pp. 647-660 ◽  
Author(s):  
Celia Zazo Seco ◽  
Luciana Serrão de Castro ◽  
Josephine W. van Nierop ◽  
Matías Morín ◽  
Shalini Jhangiani ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Unilateral Hearing Loss ◽  
Syndrome Type ◽  
Waardenburg Syndrome ◽  
Kit Ligand

Detection of Unilateral Hearing Loss by Stationary Wavelet Entropy

2017 ◽  
Vol 16 (2) ◽  
pp. 122-128 ◽  
Author(s):  
Yudong Zhang ◽  
Deepak Nayak ◽  
Ming Yang ◽  
Ti-Fei Yuan ◽  
Bin Liu ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Unilateral Hearing Loss ◽  
Wavelet Entropy

Development of receptive vocabulary and verbal intelligence in Japanese children with unilateral hearing loss

2021 ◽  
Author(s):  
Takaaki Takeyama ◽  
Aki Shimada ◽  
Yuki Sakamoto ◽  
Toshihito Aoki ◽  
Eiji Kondo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Receptive Vocabulary ◽  
Unilateral Hearing Loss ◽  
Verbal Intelligence ◽  
Japanese Children

scholarly journals Changes in auditory perceptions and cortex resulting from hearing recovery after extended congenital unilateral hearing loss

2013 ◽  
Vol 7 ◽  
Author(s):  
Jill B. Firszt ◽  
Ruth M. Reeder ◽  
Timothy A. Holden ◽  
Harold Burton ◽  
Richard A. Chole
Keyword(s):  
Hearing Loss ◽  
Unilateral Hearing Loss ◽  
Hearing Recovery
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