scholarly journals Rare Occurrence of Internal Auditory Canal Stenosis Accompanied With Congenital Facial Palsy in a 3-Month-Old Infant: A Case Report

2020 ◽  
Vol 44 (3) ◽  
pp. 256-259
Author(s):  
Se-Heum Park ◽  
Woo-Jin Kim ◽  
Yun-Jung Lim ◽  
Cheol-Won On ◽  
Ji-Ho Park ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Developmental Delay ◽  
Facial Palsy ◽  
Internal Auditory Canal ◽  
Magnetic Resonance Imaging Scan ◽  
Developmental Assessment ◽  
Canal Stenosis ◽  
The Brain ◽  
Tomography Scan

Internal auditory canal (IAC) stenosis with hypoplasia of the facial and vestibulocochlear nerves is a rare cause of congenital facial palsy. In this case report, a 3-month-old female infant was referred for a neurological developmental assessment for developmental delay and congenital facial palsy. Upon evaluation of developmental delay, hearing loss was detected. Following a magnetic resonance imaging scan of the brain and a computed tomography scan of the temporal bone, IAC stenosis with hypoplasia of facial and vestibular nerves was diagnosed. This is a rare case of IAC stenosis in an infant with initial presentations of left facial palsy and developmental delay associated with hearing loss in the left ear. We strongly suggest that IAC stenosis be considered a cause of congenital facial palsy in infants, especially in patients with developmental delay. In infants with congenital facial palsy, a thorough physical examination and neurological developmental assessment should be performed.

scholarly journals Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

2021 ◽  
pp. 014556132199683
Author(s):  
Wenqi Liang ◽  
Line Wang ◽  
Xinyu Song ◽  
Fenqi Gao ◽  
Pan Liu ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Next Generation Sequencing ◽  
Inner Ear ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Transverse Diameter ◽  
Congenital Hearing Loss ◽  
Canal Stenosis ◽  
Ear Anomalies ◽  
Generation Sequencing

The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient’s hearing was rehabilitated with bilateral cochlear implantation.

Congenital Internal Auditory Canal Stenosis With Facial Palsy

2019 ◽  
Vol 40 (2) ◽  
pp. e167-e169
Author(s):  
Tsun-Min Lu ◽  
Nai-Wei Hsueh ◽  
Lin Hung-Ching
Keyword(s):  
Facial Palsy ◽  
Internal Auditory Canal ◽  
Canal Stenosis

Syphilis causing hearing loss

2008 ◽  
Vol 19 (10) ◽  
pp. 721-722 ◽  
Author(s):  
S Y Chan ◽  
M Medhi ◽  
A Ekbote ◽  
S Moses ◽  
N Sibtain ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Highly Active Antiretroviral Therapy ◽  
Internal Auditory Canal ◽  
Secondary Syphilis ◽  
Hiv Positive ◽  
Magnetic Resonance Imaging Scan ◽  
Facial Weakness ◽  
Highly Active ◽  
Syphilis Serology ◽  
Hiv 1

An HIV-positive man with hepatitis B co-infection, naïve to highly active antiretroviral therapy, with a CD4 of 594 copies/mL and HIV-1 viral load of 140,070 copies, presented with right-sided facial weakness and hearing loss. He had been treated for secondary syphilis three months earlier when his rapid plasma reagin (RPR) result was 1:16, this had fallen to neat. At presentation, his RPR had risen to 1:16 again. A magnetic resonance imaging scan showed enhancement of the internal auditory canal and right cochlea. His cerebrospinal fluid examination was normal. He was treated with acyclovir and prednisolone before the syphilis serology was known. He was then treated for syphilis with doxycycline. He made an excellent recovery.

The Use of a Custom Earmold to Prevent Recurrent External Auditory Canal Stenosis

2008 ◽  
Vol 19 (03) ◽  
pp. 233-236 ◽  
Author(s):  
Christopher Garvey ◽  
Heather Turner
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
External Auditory Canal ◽  
Conductive Hearing Loss ◽  
Canal Stenosis ◽  
Conductive Hearing

The treatment of external auditory canal (EAC) stenoses often requires the prolonged use of a stent or splint. Traditional stents often occlude the EAC, resulting in a temporary conductive hearing loss. This case report describes a custom canal earmold with a large bore used as a stent in a patient with EAC stenosis. The customized earmold stent successfully prevented restenosis, while the large bore provided ventilation and improved hearing subjectively during the stenting phase. El tratamiento de la estenosis del conducto auditivo externo (EAC) a menudo requiere del uso prolongado de un stent o una férula. Los stents tradicionales a menudo ocluyen el EAC, provocando una hipoacusia conductiva temporal. Este reporte describe un caso un molde auditivo hecho a la medida con un agujero grande utilizado como stent en un paciente con estenosis del EAC. El stent de molde auditivo a la medida fue exitoso en prevenir la re-estenosis, mientras que el gran agujero aportó ventilación y mejoró subjetivamente la audición durante la fase de mantenimiento del stent.

Cavernous haemangioma of the internal auditory canal

2000 ◽  
Vol 114 (6) ◽  
pp. 453-455 ◽  
Author(s):  
A. M. Shaida ◽  
D. J. McFerran ◽  
M. da Cruz ◽  
D. G. Hardy ◽  
D. A. Moffat
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Facial Nerve ◽  
Early Recognition ◽  
Internal Auditory Canal ◽  
Cavernous Haemangioma ◽  
Hormonal Changes ◽  
Radiological Imaging ◽  
Radiological Findings ◽  
In Pregnancy

Cavernous haemangiomas are rare lesions of the cerebello-pontine angle that can mimic the more commonly occurring vestibular schwannoma. A case report involving a patient with a cavernous haemangioma of the internal auditory canal (IAC) highlights this as a diagnostic possibility for lesions of the IAC by comparing and contrasting the clinical and radiological findings with the more commonly occurring vestibular nerve and facial schwannomas.Symptoms such as hearing loss and facial paralysis that are disproportionate to the size of the lesion or fluctuate with hormonal changes such as those seen in pregnancy are suggestive of haemangioma. Radiological imaging demonstrating a lesion enchancing with gadolinium and containing areas of calcification is also suggestive of haemangioma. It is important to consider the possible diagnosis of haemangioma as early recognition of this entity may improve the chances of preserving the functional integrity of the facial nerve.

scholarly journals Pathogenic Variant in ACTB, p.Arg183Trp, Causes Juvenile-Onset Dystonia, Hearing Loss, and Developmental Delay without Midline Malformation

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Erin Conboy ◽  
Filippo Vairo ◽  
Darrel Waggoner ◽  
Carole Ober ◽  
Soma Das ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Developmental Delay ◽  
Brain Mri ◽  
Craniofacial Anomalies ◽  
Juvenile Onset ◽  
Caudate Nuclei ◽  
The Third ◽  
Cerebral Anomalies ◽  
The Brain ◽  
Congenital Malformation Syndrome

ACTB encodes the β-actin, and pathogenic variations in this gene have typically been associated with Baraitser-Winter cerebrofrontofacial syndrome, a congenital malformation syndrome characterized by short stature, craniofacial anomalies, and cerebral anomalies. Here, we describe the third case with the p.Arg183Trp variant in ACTB causing juvenile-onset dystonia. Our patient has severe, intractable dystonia, developmental delay, and sensorineural hearing loss, besides hyperintensities in the caudate nuclei and putamen on the brain MRI, which is a distinct but overlapping phenotype with the previously reported case of identical twins with the same alteration in ACTB.

scholarly journals Unilateral internal auditory canal stenosis with progressive facial weakness

2012 ◽  
Vol 18 (2) ◽  
pp. 179-182
Author(s):  
Sathiya Murali ◽  
Arpana Shekhar ◽  
S Shyam Sudhakar ◽  
Kiran Natarajan ◽  
Mohan Kameswaran
Keyword(s):  
Hearing Loss ◽  
High Resolution ◽  
Facial Nerve ◽  
Temporal Bone ◽  
Internal Auditory Canal ◽  
Facial Weakness ◽  
Profound Hearing Loss ◽  
Canal Stenosis ◽  
Temporal Bone Ct ◽  
Magnetic Resonance Imaging Mri

Internal auditory canal (IAC) stenosis is a rare cause of sensorineural hearing loss. Patient may present with symptoms of progressive facial nerve palsy, hearing loss, tinnitus and giddiness. High resolution temporal bone CT-scan and magnetic resonance imaging (MRI) are the important tools for diagnosis. No specific management has been devised. Here is presentation of a case of unilateral (left) IAC stenosis with profound hearing loss and progressive House Brackmann Grade III-IV facial weakness. The diameter of the IAC was less than 2 mm on high resolution temporal bone computed tomography (HRCT) scan. It was managed by facial nerve decompression by translabyrinthine approach in an attempt to prevent further deterioration of facial palsy. DOI: http://dx.doi.org/10.3329/bjo.v18i2.12014 Bangladesh J Otorhinolaryngol 2012; 18(2): 179-182

Congenital Unilateral Facial Palsy and Internal Auditory Canal Stenosis

2008 ◽  
Vol 39 (2) ◽  
pp. 116-119 ◽  
Author(s):  
Kao-Min Lin ◽  
Chao-Ching Huang ◽  
Joseph Hang Leung
Keyword(s):  
Facial Palsy ◽  
Internal Auditory Canal ◽  
Canal Stenosis
Sign in / Sign up

Export Citation Format

Share Document