A Child with Saethre-Chotzen Syndrome, Sensorineural Hearing Loss, and a TWIST Mutation

2002 ◽  
Vol 39 (1) ◽  
pp. 110-114 ◽  
Author(s):  
Samson Lee ◽  
Marianne Seto ◽  
Kathy Sie ◽  
Michael Cunningham
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Sensorineural Hearing ◽  
Twist Gene ◽  
Syndromic Craniosynostosis

Objective Patients with syndromic craniosynostosis may have associated hearing deficits. A review of hearing loss associated with syndromic craniosynostosis as well as implications of cochlear implantation in the craniosynostosis patients is presented. In the literature, patients with Saethre-Chotzen syndrome have been shown to have conductive or mixed hearing losses. This case report describes a patient with Saethre-Chotzen syndrome caused by a mutation in the TWIST gene who exhibits a severe to profound sensorineural hearing loss.

Bilateral profound sudden sensorineural hearing loss presenting a diagnostic conundrum in a child with sickle cell anaemia

2008 ◽  
Vol 123 (7) ◽  
pp. 811-816 ◽  
Author(s):  
A D Mace ◽  
M S Ferguson ◽  
M Offer ◽  
K Ghufoor ◽  
M J Wareing
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Sensorineural Hearing Loss ◽  
Sickle Cell ◽  
Cochlear Implantation ◽  
World Literature ◽  
Sickle Cell Anaemia ◽  
Early Recognition ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing

AbstractObjective:To present the first published case of a child with bilateral profound sudden sensorineural hearing loss found in association with sickle cell anaemia, and to demonstrate the importance of early recognition, investigation and empirical treatment of sudden sensorineural hearing loss.Method:Case report and review of world literature.Case report:The authors present the case of a seven-year-old child with known sickle cell anaemia, who presented with bilateral profound sensorineural hearing loss developing over a period of five days. There was a history of ophthalmological disease in the preceding weeks, and inflammatory markers were raised. The differential diagnosis included a vaso-occlusive or inflammatory aetiology such as Cogan's syndrome, and treatment for both was instigated. Hearing thresholds did not recover, and the patient underwent cochlear implantation 12 weeks later.Conclusion:Sudden sensorineural hearing loss has a variable aetiology and is rare in children. Immediate treatment for all possible aetiologies is essential, along with targeted investigations and early referral for cochlear implantation if no recovery is demonstrated.

Reversible hearing loss following cryptococcal meningitis: case study

2016 ◽  
Vol 130 (7) ◽  
pp. 691-695 ◽  
Author(s):  
W L Neo ◽  
N Durisala ◽  
E C Ho
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Bacterial Meningitis ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Cryptococcal Meningitis ◽  
Sudden Onset ◽  
Sensorineural Hearing ◽  
Labyrinthitis Ossificans

AbstractBackground:Sensorineural hearing loss is a recognised complication of cryptococcal meningitis. The mechanism of hearing loss in patients with cryptococcal meningitis is different from that in bacterial meningitis.Case report:An immune-competent man with cryptococcal meningitis presented with sudden onset, bilateral, severe to profound sensorineural hearing loss and vestibular dysfunction. He was initially evaluated for cochlear implantation. However, he had a significant recovery; he no longer required surgery and was able to cope without a hearing aid.Conclusion:Typically, cochlear implantation is performed with some urgency in patients with hearing loss post-bacterial meningitis, because of the risk of labyrinthitis ossificans. However, this process has not been described in patients with cryptococcal meningitis. Furthermore, patients with hearing loss associated with cryptococcal meningitis have shown varying degrees of reversibility. In this case report, hearing loss from cryptococcal meningitis is compared with that from bacterial meningitis, and the need for cochlear implantation in patients with cryptococcal meningitis is discussed.

scholarly journals Cochlear Implantation Following Explorative Tympanotomy in Patients With Sudden Sensorineural Hearing Loss: Surgical Features and Audiological Outcomes

2021 ◽  
pp. 014556132110091
Author(s):  
Robin Rupp ◽  
Joachim Hornung ◽  
Matthias Balk ◽  
Matti Sievert ◽  
Sarina Müller ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Connective Tissue ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Round Window ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Round Window Membrane ◽  
Hearing Results ◽  
Round Window Niche

Objective: To investigate the anatomical status of the round window niche and hearing outcome of cochlear implantation (CI) after explorative tympanotomy (ExT) with sealing of the round window membrane in patients with sudden sensorineural hearing loss at a tertiary referral medical center. Methods: Between January 1, 2007, and July 30, 2020, 1602 patients underwent CI at our department. Out of these, all patients previously treated by ExT with sealing of the round window membrane because of unilateral sudden hearing loss were included in the study. A retrospective chart review was conducted concerning method of round window membrane sealing, intraoperative findings during CI, postoperative imaging, and hearing results. Results: Twenty one patients (9 females; 8 right ears; 54.3 years [± 12.9 years]) underwent ExT with sealing of the round window membrane with subsequent CI after 26.6 months (± 32.9 mo) on average. During CI, in 76% of cases (n = 16), the round window niche was blocked by connective tissue due to the previous intervention but could be removed completely in all cases. The connective tissue itself and its removal had no detrimental effects on the round window membrane. Postoperative computed tomography scan showed no electrode dislocation. Mean postoperative word recognition score after 3 months was 57.4% (± 17.2%) and improved significantly to 73.1% (± 16.4%, P = .005) after 2 years. Conclusion: Performing CI after preceding ExT, connective tissue has to be expected blocking the round window niche. Remaining tissue can be removed safely and does not alter the round window membrane allowing for a proper electrode insertion. Short- and long-term hearing results are satisfactory. Consequently, ExT with sealing of the round window membrane in patients with sudden sensorineural hearing loss does not impede subsequent CI that can still be performed safely.

Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis

2013 ◽  
Vol 127 (7) ◽  
pp. 708-711 ◽  
Author(s):  
A C Hall ◽  
A C Leong ◽  
D Jiang ◽  
A Fitzgerald-O'Connor
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Skin Lesions ◽  
Sudden Hearing Loss ◽  
Sudden Onset ◽  
Sensorineural Hearing ◽  
Urticarial Vasculitis ◽  
Wells Syndrome ◽  
Bilateral Sensorineural Hearing Loss

AbstractBackground:Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle–Wells syndrome. Previous reports have described the hearing loss to be progressive in nature.Method:To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle–Wells syndrome.Results:The patient underwent a cochlear implantation with a modest outcome.Conclusion:Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

Metronidazole ototoxicity - report of two cases

1999 ◽  
Vol 113 (4) ◽  
pp. 355-357 ◽  
Author(s):  
S. M. Iqbal ◽  
J. G. Murthy ◽  
P. K. Banerjee ◽  
K. A. Vishwanathan
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Oral Administration ◽  
Sensorineural Hearing Loss ◽  
Steroid Therapy ◽  
World Literature ◽  
Sensorineural Hearing ◽  
Oral Steroid ◽  
The World ◽  
Oral Steroid Therapy

AbstractTwo cases of bilateral moderate to severe sensorineural hearing loss due to oral administration of metronidazole are reported. There has been only one case report of deafness following metronidazole therapy in the world literature. The hearing loss recovered gradually in a period of four to six weeks following withdrawal of drug and oral steroid therapy. The possible mechanism of ototoxicity is discussed. Awareness by the treating physician of ototoxicity due to any drug is stressed.

scholarly journals Alport Syndrome: The Eye as a Window to the Human Body

2021 ◽  
Vol 33 (3) ◽  
pp. 46-48
Author(s):  
Aysha Tareq Nusef ◽  
Abdulla Almoosa ◽  
Wael Wagih Aly
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Renal Insufficiency ◽  
Sensorineural Hearing Loss ◽  
Alport Syndrome ◽  
Crystalline Lens ◽  
Secondary Hypertension ◽  
Sensorineural Hearing ◽  
Blurred Vision ◽  
Ophthalmic Manifestations

Alport syndrome (AS) is a rare genetic disease affecting type four collagen production, causing renal, auditory, and ophthalmic manifestations. This case report is about a 32-year-old male who was a known case of renal insufficiency and secondary hypertension and was referred to the ophthalmology department due to blurred vision. Based on the patient‘s history and ophthalmological findings, AS was diagnosed. Ophthalmic examination showed anterior lenticonus associated with sensorineural hearing loss (SNHL) and impaired renal function. This clinical case report sheds light on the role of ophthalmology in diagnosing AS. Keywords: Collagen, Crystalline lens, Hereditary nephritis, Ophthalmology, Renal insufficiency, Sensorineural hearing loss

scholarly journals Superficial Siderosis and Sudden Sensorineural Hearing Loss: A Case Report and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-2
Author(s):  
Kirsti S. V. Lee ◽  
Niranjan Sritharan ◽  
Allan Forrest
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Sensorineural Hearing Loss ◽  
Unusual Case ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Superficial Siderosis ◽  
Imaging Features ◽  
Delayed Complication ◽  
Magnetic Resonance Imaging Mri

This case report highlights an unusual case of sudden sensorineural hearing loss related to superficial siderosis (SS). Our patient had a craniotomy for medulloblastoma 23 years earlier, and this may represent a delayed complication related to this procedure. Magnetic resonance imaging (MRI) remains the key diagnostic investigation to illustrate the imaging features of superficial siderosis and exclude other pathologies. Increased awareness of progressive and sudden hearing complications caused by SS is important in the otolaryngologic community to expedite management and better counsel patients during the consent process.

Imaging case study: enlarged superior vestibular nerve canal with sensorineural hearing loss

2008 ◽  
Vol 123 (5) ◽  
pp. 572-574 ◽  
Author(s):  
M I Redleaf ◽  
J M Pinto ◽  
J J Klemens
Keyword(s):  
Computed Tomography ◽  
Hearing Loss ◽  
Case Report ◽  
Sensorineural Hearing Loss ◽  
Temporal Bone ◽  
Male Infant ◽  
Vestibular Nerve ◽  
Sensorineural Hearing ◽  
Inferior Vestibular Nerve

AbstractObjective:We report a new temporal bone anomaly – an enlarged superior vestibular nerve canal – associated with sensorineural hearing loss.Case report:A 10-month-old male infant presented with sensorineural hearing loss together with bilaterally enlarged superior vestibular nerve canals. Compared with published temporal bone computed tomography measurements, our patient's canals were normal in length but approximately double the normal width. In addition, careful review of the imaging did not clearly identify a bony wedge between the superior and inferior vestibular nerve canals.Conclusion:Enlarged superior vestibular nerve canal malformation may be a marker for sensorineural hearing loss. Increased vigilance amongst otologists may establish the prevalence of this anomaly and its possible effects on hearing.

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