Bilateral Mondini Research Articles

Hemifacial microsomia and bilateral Mondini dysplasia

We present a rare association of bilateral Mondini dysplasia with hemifacial microsomia (HFM). HFM is a clinical spectrum of malformations involving the orbit, mandible, ears, facial nerve, and soft tissues of the face. It occurs in about 1 in 5600 births and requires a multidisciplinary approach to management.[1] We report a case of HFM in an 11-year-old girl who presented with recurrent discharge from the nasal dorsum, and facial asymmetry since birth but was otherwise asymptomatic. The hearing evaluation revealed bilateral sensorineural hearing loss. High-resolution computed tomography scan of the temporal bones showed bilateral Mondini dysplasia of the inner ear which to the best of our knowledge has not been previously reported in HFM. We conclude that all cases of HFM regardless of symptomatology should undergo hearing evaluation. This will facilitate rehabilitation of deafness if present.

Three Cases of Cochlear Implants in Mondini Dysplasia

Mondini dysplasia is a kind of cochlear malformation caused by a failure growth of cochlea in the seventh week of pregnancy, results in cochlear turn which only reaches 1,5 turn until less than 2,5 turn. There is sensorineural hearing loss in Mondini dysplasia caused by anatomical malformation. The diagnosis of Mondini dysplasia is made by accurate anamnesis, audiology assessments and imaging results. This case study explains the importance of the holistic process of diagnosis and treatment of Mondini's dysplasia. Purpose: To explain that Mondini dysplasia should be diagnosed immediately and given the cochlear implant. Cases: There had been reported three cases of cochlear malformation of Mondini dysplasi type. They were first diagnosed with sensorineural hearing loss and speech delay in the age of 2-4 years. After had been done sequence of audiology assessments and imaging CT-scan and MRI simultaneously there were obtained that those three patients had Mondini dysplasia. Case Management: The use of hearing aid in those three patients had no effectiveness that brought them to the decision of cochlear implantation. Cochlear implant became such a great challenge even through for experienced physicians considering the scarcity of the case and the risk for complications like pelymph gusher and incorrect insertion of electrode. Cochlear implant surgeries had been done to those three patients with carefu consideration and the selection for appropriate type of electrode. On the first case there had been applied right ear cochlear implant, on the second case was bilateral, and on the third case was in the left ear. Complications of the surgeries were bleeding and perilymph gusher but they had been solved properly. Electrode insertion in all three cases had been placed correctly. Conclusion: Found three cases of Mondini dysplasia with symptoms of bilateral hearing loss, speech development, bilateral Mondini dysplasia, techniques and complications of cochlear implants. These three cases differ in the shape, width and size of the cochlear anatomy if they are studied more specifically. Two cases of unilateral cochlear implant placement and one bilateral case.

Mondini Deformity in a Case of Turner Syndrome. A Radiological Finding

Turner syndrome (TS) is the human being's most frequent sex chromosome abnormality. Progressive sensorineural hearing loss is documented in more than 50% of the women affected by this syndrome. Although Mondini defect is the cochlear congenital malformation most frequently identified in other polymalformative syndromes, it has rarely been reported in TS. We describe the case of a 32-year-old woman with TS who presented progressive sensorineural hearing loss. The computed tomography of the ears showed bilateral Mondini deformity.

Bilateral enlarged vestibular aqueduct with associated bilateral Mondini's dysplasia

Enlarged Vestibular Aqueduct (EVA) and Mondini's dysplasia (incomplete partitioning type II) are entitites that have been fairly well described in the literature as potential causes of hearing loss in the young. However, it is uncommon for this condition to be detected bilaterally, especially so for both conditions to coexist bilaterally in the same patient. This is a brief description of a patient with the above bilateral condition with attached high resolution CT scan images of the temporal bone to illustrate the case.

Bilateral Mondini dysplasia associated with recurrent meningitis

Bilateral Mondini dysplasia associated with recurrent meningitis

Deformidad de Mondini en un caso de síndrome de Turner. Un hallazgo radiológico

Turner syndrome (TS) is the human being's most frequent sex chromosome abnormality. Progressive sensorineural hearing loss is documented in more than 50% of the women affected by this syndrome. Although Mondini defect is the cochlear congenital malformation most frequently identified in other polymalformative syndromes, it has rarely been reported in TS. We describe the case of a 32-year-old woman with TS who presented progressive sensorineural hearing loss. The computed tomography of the ears showed bilateral Mondini deformity.

Group B Streptococcus Meningitis in a Child with Cochlear Implant

Group B Streptococcus Meningitis in a Child with Cochlear Implant

Identification of a novel mutation in the SLC26A4 gene in an Italian with fluctuating sensorineural hearing loss

Pendred syndrome is an autosomal recessive disorder characterized by congenital sensorineural deafness, goitre and defective iodide organification. Congenital and profound hearing loss is the hallmark of the syndrome, while goitre and thyroid dysfunction are highly variable even within the same family. Clinical features are due to altered formation of pendrin, a chloride/iodide transporter protein expressed in the inner ear, thyroid gland and kidney. A novel substitution was found in exon 7 of the pendrin encoding gene ( SLC26A4) that leads to a stop codon, S314X. The new variation was found in compound heterozygosity with L445W mutation in a hearing impaired patient with bilateral Mondini's dysplasia and goitre.

Composition of the Endolymphatic Sac Luminal Fluid in a Patient with Mondini Dysplasia

We determined the chemical composition of the human endolymphatic sac luminal fluid in a patient with Mondini dysplasia. Four milliliters of endolymphatic sac luminal fluid was sampled in a 41-year-old patient who presented with bilateral Mondini dysplasia and underwent auditory brain stem implantation. Ion and protein concentrations, as well as the osmolarity of the fluid, were analyzed by means of biochemical automated procedures. The sodium, potassium, and protein concentrations and the osmolarity of the sample were 87 mmol/L, 24 mmol/ L, 33.5 g/L, and 258 mOsm/L, respectively. The composition of the endolymphatic sac luminal fluid found in our patient was similar to that determined in guinea pigs under physiological conditions, and very different from the usual composition of the cochleovestibular endolymph. Contamination of the cochleovestibular endolymphatic compartment by this sodium-rich, potassium-poor, and hypo-osmotic fluid might impair cochleovestibular function by altering the chemical composition of the cochleovestibular endolymph.

Intrathecal Fluorescein to Localize Cerebrospinal Fluid Leakage in Bilateral Mondini Dysplasia

Recurrent meningitis caused by bilateral Mondini dysplasia presents a difficult diagnostic and therapeutic dilemma. A high-resolution computed tomographic scan with intrathecal contrast provided a false-positive identification for the side of cerebrospinal fluid leak in this patient. We report the intraoperative use of intrathecal fluorescein with mastoid tap to identify the side of cerebrospinal fluid leak in a 16-month-old boy with bilateral Mondini malformations. Both ears had been obliterated for recurrent cerebrospinal fluid leaks, while meningitis occurred after unilateral cochlear implantation.

Tekrarlayan streptococcus pneumoniae menenjiti ve Mondini displazisi: olgu sunumu Olgu Sunumu

Mondini dysplasia is a congenital malformation of the middle ear commonly associated with hearing impairment otorrhea rhinorrhea and recurrent meningitis In this report a 13 year old girl with recurrent meningitis due to Streptococcus pneumoniae and sensorineural hearing loss is presented Bilateral Mondini dysplasia was demonstrated with computed tomography scans of the temporal bones Mondini dysplasia should be considered in patients with recurrent meningitis and deafness Key words: Mondini dysplasia recurrent meningitis

Congenital aplasia of the semicircular canals.

To describe the underrecognized inner ear malformation characterized by complete aplasia of the labyrinthine semicircular canals associated with a relatively well-formed cochlea, to investigate its relationship with known syndromic forms of hearing loss, and to hypothesize regarding the potential embryopathogenesis of this anomaly. A retrospective case review consisting of cases of sensorineural hearing loss with radiographic evidence demonstrating agenesis of the semicircular canals associated with a cochlea that was either morphologically normal or sufficiently well developed to accommodate the full insertion of a cochlear implant electrode. Cases were identified by computerized tomography findings that identified the anomaly under study. Departments of otolaryngology and radiology in a tertiary referral center, with a large cochlear implant program serving over 800 patients, more than half of whom are children. Fifteen patients with the anomaly under study were identified. Each patient underwent a complete otologic examination, audiometric studies, and high resolution computerized tomography of the temporal bone in axial and coronal planes. The bony morphology of the cochlea, round and oval windows, vestibule, semicircular canals, and vestibular aqueduct, and the course of the facial nerve were examined. Auditory findings and otologic treatment are presented. Of the 15 identified patients, 4 were nonsyndromic, 9 had CHARGE association (Coloboma of the eye, congenital Heart defects, choanal Atresia, mental and/or growth Retardation, Genital hypoplasia, and Ear anomalies and/or deafness), 1 met criteria for Noonan's syndrome, and one had features of both these syndromes. Although the cochlea was present in all cases, the cochlear morphology was usually abnormal in the CHARGE association patients. Of the 20 ears in the CHARGE subjects, only 3 ears (15%) were seen to have completely normal development of the cochlea in both the basal and upper turns. The others showed either mild hypoplasia of the upper turns (13 ears, 65%) or an incomplete partition typical of the classic Mondini deformity (4 ears, 20%). In the 4 nonsyndromic cases, one subject had bilateral Mondini dysplasia and the other three had normal cochleae. In the entire group, abnormalities of oval window development were common (20 of 30 ears, 67%), especially in the syndromic cases (18/22, 81%), but the round window was normal in the majority of cases (73%). Seven patients in the CHARGE association group had an anomalous course of the facial nerve, which was particularly severe in three. Four patients had congenital unilateral facial paralysis, although two of these children had normal radiographic anatomy of the facial nerve. One patient had bilateral facial weakness. Syndromic and nonsyndromic cases of isolated semicircular canal aplasia were identified. Except for mild to moderate cochlear dysplasia, and the anomalous course of the facial nerve in some CHARGE association patients, both groups of patients were generally suitable for cochlear implantation if indicated. A high incidence of oval window aplasia with normal round window development may help to explain the embryopathogenesis of this anomaly, considering the sequence of inner ear development.

Mondini dysplasia and congenital cytomegalovirus infection

We report a case of bilateral temporal bone anomalies in a child with symptomatic congenital cytomegalovirus infection and severe, bilateral sensorineural hearing loss identified at 3 months of age. High-resolution temporal bone computed tomography (HRCT) revealed bilateral findings of a short, malformed cochlea lacking an interscalar septum, a short and wide internal auditory canal, and an enlarged vestibular aqueduct, features diagnostic of bilateral Mondini dysplasia. To determine the importance of this observation, we completed HRCT in five additional children between 7 months and 9 years of age who had evidence of symptomatic congenital cytomegalovirus infection. One child with profound sensorineural hearing loss had severe bilateral temporal bone dysplasia with a small cochlea lacking an interscalar septum, an abnormal vestibule, and a large cochlear aqueduct. Of the remaining four children, hearing thresholds ranged from normal to profoundly decreased, but their HRCT scans were normal to visual inspection. When inner ear dimensions of these temporal bones were compared with norms established by Pappas and co-workers, however, seven of the eight ears had short cochleas and narrow lateral semicircular canals, and three ears had short or narrow vestibules. These results indicate that congenital cytomegalovirus infection may cause anomalies or growth disturbances of the temporal bone. (J P EDIATR 1994;124:71-8)

一家系に多発した両側性Ｍｏｎｄｉｎｉ型内耳奇形

High resolution computed tomography (HRCT) of the temporal bone has enabled us to diagnose inner ear anomalies easily. Otorhinolaryngologists will be able to diagnose Mondini type malformation more frequently. A 55-year-old female visited us because of hearing loss and vertigo. Pure tone audiograms revealed bilateral deafness. Bilateral Mondini type inner ear malformations were observed in the HRCT of the temporal bone. Her family history revealed that there were five women with severe hearing impairment. We perform temporal bone HRCT and audiograms on three women with impaired hearing and a woman with normal hearing in her family. Pure tone audiograms of three women revealed nearly bilateral deafness and temporal bone HRCT showed bilateral Mondini type inner malformations similar to one another. The CT of the woman with normal hearing showed completely normal findings. Autosomal dominant inheritance was suspected because of the family history.