Auditory manifestations of superficial hemosiderosis of the central nervous system

Denis, Arnaud; Blaivie, Cathérine; Kohen, A. El; Tosello, Lucrecia; Williams, Marc T.

doi:10.1007/s00405-006-0238-0

Cited by 11 publications

(4 citation statements)

References 16 publications

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“…The high vulnerability of the eighth nerve is thought due to its long glial segment and the fact that the eighth nerve passes through the pontine cistern which has a greater flow of CSF than for other cranial nerves (1). A progressive, high frequency sensorineural hearing loss has been described in a number of case reports (6-8). …”

Section: Introductionmentioning

confidence: 99%

Temporal Bone Histopathology in a Case of Sensorineural Hearing Loss Caused by Superficial Siderosis of the Central Nervous System and Treated by Cochlear Implantation

Nadol¹,

Adams²,

O’Malley³

2011

Otology &Amp; Neurotology

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Objectives To evaluate the histopathology of the temporal bones of a patient with documented superficial siderosis of the central nervous system who underwent right cochlear implantation six years before death. Background Superficial siderosis of the central nervous system is due to chronic or repeated subarachnoid hemorrhage and results in sensorineural deafness in 95% of affected individuals in addition to other neurologic findings. The deposition of hemosiderin in the meninges and around cranial nerves is thought to be causative. There have been no previous reports of temporal bone pathology in this disorder. This 57 year old man developed progressive, bilateral hearing loss starting in his 30's with loss of pure tone thresholds and word recognition. He underwent a right cochlear implant at age 51 with full insertion of the device. Methods The temporal bones and brainstem were fixed in formalin and prepared for histologic study by standard techniques. Special stains, including Gomori stain for iron were performed on sections of the temporal bones and cochlear nucleus. Results There was severe bilateral degeneration of the organ of Corti, spiral ligament, stria vascularis, and spiral ganglion cells. Gomori stain revealed iron deposits within the spiral ligament, stria vascularis and in the subepithelial mesenchymal tissue of the maculae of the vestibular system. Evaluation of the cochlear nucleus revealed iron deposits within glial cells and larger cells, probably macrophages, near the CSF surface. On the right side, the track created by the cochlear implant entered the scala tympani and continued to mm17, as measured from the round window. Discussion and Conclusions This is the first known case of superficial siderosis with documented temporal bone histopathology. Hearing loss was likely caused by severe degeneration of spiral ganglion cells in both ears, despite the presence of remaining hair cells in the middle and apical turns. This was consistent with cochlear neuronal degeneration and retrograde degeneration of spiral ganglion cells within the inner ear, or alternatively, consistent with primary degeneration of hair cells and neural structures within the cochlea. Despite the presence of neural degeneration, the patient achieved a word recognition score of 28% six months following implantation.

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Section: Introductionmentioning

confidence: 99%

Temporal Bone Histopathology in a Case of Sensorineural Hearing Loss Caused by Superficial Siderosis of the Central Nervous System and Treated by Cochlear Implantation

Nadol¹,

Adams²,

O’Malley³

2011

Otology &Amp; Neurotology

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“…In our series of three patients with SHL associated with SH-CNS, only 1/3 complained of cerebellar ataxia and 2/3 had loss of olfactory hearing. SHL in SH-CNS affects high-tone frequencies at the beginning and progresses slowly to a profound SHL over a period of many years [6,13,14]. A rapid progression of SHL was also described [1,7,8,13,15].…”

Section: Discussionmentioning

confidence: 97%

Cochlear implantation in patients affected by superficial hemosiderosis of the central nervous system

Berrettini

Vito

Bruschini

et al. 2011

Eur Arch Otorhinolaryngol

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The objectives of the study were to: report three more cases of cochlear implantation (CI) in patients affected by superficial hemosiderosis of the central nervous system (SH-CNS); assess whether CI may be a viable option in this disease. The study was conducted in a tertiary referral center. Pre-operative and post-operative clinical notes of three patients with SH-CNS were reviewed. Two out of three cases showed very good results with CI in sentence perception in noise over 90%. For the other case, hearing performance was very low. He showed only disyllabic word identification abilities in a closed set (40%). Cochlear implantation may be a viable option for patients with severe to profound sensorineural hearing loss due to SH-CNS. In these cases, an adequate pre-operative counseling, explaining the possibility of poor post-operative results and/or the worsening of the outcomes in the following years, is important.

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“…However, in rare cases, a central origin such as an acoustic tumor or infarction of the anterior inferior cerebellar artery might be the cause 9) . In rare cases, superficial hemosiderosis, cytomegalovirus, borreliosis, radiation therapy, neurosyphilis, non-hodgkin's lymphoma, malignant melanoma and paraneoplastic cerebral syndrome might cause hearing loss [1][2][3]5,10,11,14) . However, hearing loss as the presenting symptom of an intracerebral hemorrhage is very rare; it may be overlooked because patients usually present with disabilities such as hemiplegia, paresthesia, gait disturbance, and dizziness, which are more commonly associated with pathology of a central origin 12) .…”

Section: Discussionmentioning

confidence: 99%

A Case of Temporal Intracerebral Hemorrhage That Presented with Sudden Bilateral Hearing Loss as the Initial Symptom

Mun

Hong

Kang

et al. 2010

J Korean Neurosurg Soc

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J Korean Neurosurg Soc 48 : [438][439][440] 2010 A 57-year-old man presented to the outpatient department with sudden bilateral hearing loss. The otological examination suggested bilateral severe sensorineural hearing loss. After several hours, the patient complained of a headache and became drowsy. The brain computed tomography showed a 3 x 4 cm intracerebral hemorrhage (ICH) of the left temporal lobe. Surgery was performed and 34 days after the procedure the patient was discharged from the hospital with severe bilateral sensorineural hearing loss (SNHL). Temporal lobe ICH should be considered in the differential diagnosis of patients with sudden bilateral hearing loss, regardless of the other neurological symptoms. KEY WORDS :Intracerebral hemorrhage˙ Sudden hearing loss˙ Temporal lobe.

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Auditory manifestations of superficial hemosiderosis of the central nervous system

Cited by 11 publications

References 16 publications

Temporal Bone Histopathology in a Case of Sensorineural Hearing Loss Caused by Superficial Siderosis of the Central Nervous System and Treated by Cochlear Implantation

Temporal Bone Histopathology in a Case of Sensorineural Hearing Loss Caused by Superficial Siderosis of the Central Nervous System and Treated by Cochlear Implantation

Cochlear implantation in patients affected by superficial hemosiderosis of the central nervous system

A Case of Temporal Intracerebral Hemorrhage That Presented with Sudden Bilateral Hearing Loss as the Initial Symptom

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