Rabia Shihada

Acute mastoiditis: the role of imaging for identifying intracranial complications.

Brain CT is performed in patients presenting with acute mastoiditis (AM) to identify intracranial complications (ICC). Recently, however, the need for CT scans in such patients has been questioned owing to concerns regarding long‐term effects of brain irradiation, with some clinicians claiming that the decision to scan should be based on a patients clinical presentation. This study was aimed at characterizing the typical clinical presentation of patients who already have ICCs when diagnosed with AM, and to compare it to that of AM patients presenting without ICCs.

Endovascular embolization of a hemorrhagic jugular bulb diverticulum

A jugular bulb diverticulum (JBD) is considered to be a rare venous anomaly. In exceptional cases it extends into the middle ear cavity with no bony covering, and slight oozing to massive bleeding might be encountered during routine middle ear surgical procedures. We report a case of massive bleeding from a dehiscent jugular bulb diverticulum that appeared during myringotomy and was successfully managed by endovascular embolization. This report emphasizes the value of interventional neuroradiology in otosurgical cases in which preoperative control of blood vessels or vascular malformations is crucial to avoid severe complications.

Laryngeal Mass with Multiple Cranial Neuropathies as a Presenting Sign for Varicella Zoster Infection

The most common presentation of varicella zoster virus (VZV) infection is unilateral distribution of herpetic eruptions and neuralgia. Laryngeal involvement is considered very rare.

Unilateral Blindness following Septoplasty

Visual loss following routine nasal surgery is an extremely rare and devastating complication. We report a case of unilateral blindness due to orbital apex syndrome following septoplasty. We also review the literature and discuss probable causes. To the best of our knowledge, this is only the second published report of this complication.

Spontaneous regression of petrous apex cholesterol granuloma.

Cholesterol granuloma (CG) is the most common pathologic lesion of the petrous apex (PA) (1,2). The lesion is thought to result from a foreign-body giant cell reaction in conjunction with fibrosis, vascular proliferation, and the presence of hemosiderin-laden macrophages. Symptoms vary depending on the size and site of the lesion and may include hearing loss, vertigo, headaches, tinnitus, change in facial sensation, otalgia, diplopia, trigeminal neuralgia, facial spasm, and seizure (2). Surgical treatment is indicated for symptomatic and/or growing lesions. A 46-year-old man presented to our department in 2007 after experiencing mild intermittent left hemicranial headaches for 2 months. He also reported having leftsided mild tinnitus for several years. Physical examination, including neurotologic assessment, was normal. There were no cranial nerves deficits and no complaints of diplopia. Audiometry revealed bilaterally symmetric mild high-tone sensorineural hearing loss. Auditory brainstem response showed normal wave patterns. Computed tomography (CT) revealed an expansile lesion filling the left PA, with erosion of the carotid canal wall (Fig. 1A). MRI showed a hyperintense signal on both T1and T2-weighted sequences (Fig. 1, B and C), characteristics typical of PA CG. Because indications for surgical treatment of PA CG are a symptomatic or a growing lesion and our patient’s symptoms were only intermittent headache and mild tinnitus, both possibly unrelated to the lesion, we opted to consider his PA CG as questionably symptomatic and to adopt a ‘‘wait-and-scan’’ strategy. Two years later, repeated MRI scan showed that the lesion had significantly decreased in size, was less hyperintense, and contained an aerated area inside of it (Fig. 2). At follow-up examination, the patient stated that his complaints completely subsided. CG often is reported in the PA, middle ear, frontal sinus, mastoid (2), pterygoid process of the sphenoid sinus (3), and the maxillary sinuses (4). In addition to CG, diagnosis of PA lesions includes effusion, cholesteatoma, chondroma, chondrosarcoma, arachnoid cyst, and vascular malformation. On CT, CG is nonenhancing, well marginated, and isodense with brain. Bone erosion is usually present. MRI is pathognomonic, with high signal intensity on both T1and T2-weighted images but without gadolinium enhancement (3). The accepted management strategies for PA CG are either surgical treatment or a ‘‘wait-and-scan’’ policy. A surgical approach is selected on the basis of hearing status and lesion anatomy (3). Given that asymptomatic nongrowing lesions require no intervention, the nonsurgical approach is recommended for asymptomatic patients who are diagnosed incidentally by imaging, for symptomatic patients whose general health condition is poor, or for elderly patients. Long-term follow-up with yearly CT/MRI is advisable in such cases, as rapid growth can occur after years of stability (5). The significant regression demonstrated on MRI at the 2-year follow-up examination in our patient was surprising, and we were unable to find any published reports of spontaneous regression of PA CG or of PA lesions with similar imaging characteristics. The unexpected finding raised the possibility that this PA lesion might not be a CG but a different lesionwith similarMRI features. The narrow differential diagnostic spectrum of such lesions includes diploic fatty marrow, which is an asymmetrically nonpneumatized or less pneumatized PA characterized by a high T1-weighted signal and typically intermediate T2weighted signal on fast-spin echo. Another possibility is that a simple PA effusion, secondary to otitis media, is obstructing the air cell tracts connecting the middle ear to the PA. Unlike CG, both these lesions are nonexpansile and nondestructive, demonstrating preserved bone trabeculae on CT (2,4). Address correspondence and reprint requests to Michal Luntz, M.D., Department of OtolaryngologyYHead and Neck Surgery, Bnai Zion Medical Center, Technion-Israel Institute of Technology, P.O. Box 4940, 31048 Haifa, Israel; E-mail: michal.luntz@b-zion.org.il The authors declare no conflicts of interest. Otology & Neurotology 33:e9Ye1

Cochlear implantation in a patient with paget's disease

Hearing loss in Pagets disease, a metabolic bone disease, has been reported in as many as 60% of cases, and is presumably related to changes in mineral density of the otic capsule. We describe a 59‐year‐old man with Pagets disease who had severe to profound hearing loss and was referred to us for cochlear implantation. Preoperative evaluation revealed poor communication skills despite significant residual hearing, raising concerns about postimplantation outcome. Nevertheless, implantation was successful. As our literature search yielded only one report of cochlear implantation in Pagets disease, we record our clinical experience and discuss the hearing rehabilitation dilemmas in this case.

Coexisting middle ear cholesteatoma and giant petrous apex cholesterol granuloma.

*Department of OtolaryngologyYHead and Neck Surgery, Bnai Zion Medical Center, Technion-Israel Institute of Technology, Haifa; ÞDepartment of Radiology, Tel-Aviv Sourasky Medical Center, Tel-Aviv University, Tel-Aviv; þDepartment of OtolaryngologyYHead and Neck Surgery, Carmel Medical Center, Technion-Israel Institute of Technology, Haifa, Israel; and §Department of OtolaryngologyYHead and Neck Surgery, and Neurological Surgery, University of Southern California School of Medicine, House Ear Clinic, Los Angeles, California, U.S.A.

Group B Streptococcus Meningitis in a Child with Cochlear Implant

To the Editor: Streptococcus agalactiae, designated group B streptococcus (GBS), is a major cause of infections in neonates and young infants (1). Invasive GBS disease in children beyond infancy is uncommon, occurring mainly as bacteremia without a focus; meningitis caused by GBS is rarely reported (2). Cochlear implant recipients have been documented as having a higher rate of postimplantation bacterial meningitis than a cohort of the same age in the general US population (3). However, no cochlear implant recipient described has been reported to be infected with GBS. We report a case of GBS meningitis in a 6-year-old boy with a cochlear implant. The patient was hospitalized in 2007 with a 1-day history of fever, headache, and vomiting. His medical history indicated congenital bilateral deafness diagnosed at 1 month of age and consistent with Patterson syndrome (i.e., unusual facies, deafness, bronzed hyperpigmentation of the skin, cutis laxa, mental retardation, and bony deformities) (4). At 4 years of age, he received a right-ear cochlear implant with good functional result. Preoperative high-resolution computed tomography of the temporal bones showed bilateral inner ear malformations of both the cochlear and vestibular labyrinth, conditions consistent with bilateral Mondini deformity (5). Mastoids and middle ears were well aerated. No evidence of cerebrospinal fluid leak appeared during physical examination or imaging. He received a dose of 23-valent pneumococcal polysaccharide vaccine. At the time of hospital admission, he was somnolent but could be aroused and was cooperative. He had nuchal rigidity, dysmorphic facies, and oligodactyly. Fundi, skin, and ears were unremarkable on examination. Lumbar puncture showed a total protein level of 204 mg/dL, a glucose level of 1.6 mmol/L (blood glucose 3 mmol/L), and 4,800 leukocytes/mm3 with 88% neutrophils; no bacteria were seen on the Gram stain. Blood count was remarkable for leukocytosis of 30,000/mm3 and neutrophil predominance. The patient received treatment with dexamethasone, vancomycin, and ceftriaxone; after treatment, his condition improved rapidly. Blood culture was sterile, but GBS grew in the cerebrospinal fluid culture (the isolate being resistant only to tetracycline). Therapy was continued with ampicillin for 3 weeks. Repeated testing of his hearing and speech perception with the cochlear implant showed no deterioration. GBS plays a major role in early- and late-onset infections in neonates and young infants (1). Infections in older children and adults have been described, especially in elderly patients or those suffering from chronic conditions such as diabetes mellitus, malignancy, or HIV infection (6). A review of medical records of patients with GBS infections over a 7-year period at a children’s hospital in Memphis, Tennessee, USA, showed that, among 18 patients >3 months of age (13% of all GBS infections in the study), bacteremia was most commonly reported; 3 cases of ventriculo-peritoneal shunt infections were recorded, but no cases of meningitis without foreign devices were found (2). GBS meningitis in children beyond infancy is rare; only a few cases have been reported (7). Cochlear implantation is the standard treatment for children and adults affected by severe and severe-to-profound sensorineural hearing loss. The implant is a neural stimulator with an electrode array surgically placed near the auditory nerve fibers in the scala tympani of the cochlea. Pediatric cochlear implant recipients were found to be at higher risk for developing bacterial meningitis than children in the general US population (3). Increased risk was evident in the perioperative period but extended to >2 years postimplantation (8). Most meningitis cases were associated with an implant with a positioner, a silastic wedge inserted next to the implanted electrode in the cochlea to position the electrode closer to the cochlear nerve endings and thus facilitate electrical signal transmission. Most of those infections were caused by Streptococcus pneumoniae, and none by GBS (3,8). In our patient, the implant did not include a positioner. The timing of meningitis was consistent with the timing indicated in previous reports, but the infecting organism was unique. Inner ear malformations themselves are associated with increased risk for meningitis (9). The patient reported here had bilateral inner ear malformations; therefore, estimating the relative role of the deformity compared with the cochlear implant’s role in the pathogenesis of meningitis in his case is difficult. Meningitis in patients with inner ear malformations is associated with bacteria (e.g., S. pneumoniae and Haemophilus influenzae) that colonize the upper airways. The prevalence of oropharyngeal colonization with GBS is low (≈5%), explaining the rarity of GBS meningitis (10). Unlike for pneumococcal meningitis, which can be prevented at least partially by vaccination, no vaccine is available for GBS. Our report adds another example to the growing spectrum of invasive GBS disease beyond infancy. GBS is uniformly susceptible to penicillin; therefore, treatment directed at common causes of bacterial meningitis is also appropriate for GBS (1,10). Cochlear implant recipients with symptoms of fever, otitis media, or headache should be carefully assessed; if meningitis is diagnosed, GBS should be considered as a possible causative organism.

Severe complicated mastoiditis caused by nontypable Haemophilus influenzae

Non-typable Haemophilus influenzae (NTHi) is emerging as an important cause of invasive disease in immunized children. We describe a healthy, fully immunized 4-year old child who presented with bacteremia due to NTHi without overt acute otitis media (AOM), and subsequently developed severe mastoiditis complicated by sigmoid sinus thrombosis. Although, mastoiditis is typically regarded as a complication of AOM, those cases without antecedent AOM may represent an entirely different pathophysiological process with NTHi bacteremia seeding the mastoid. We present another example of the potential invasiveness of NTHi with a temporal sequence of events whereby NTHi bacteremia seeds the mastoid. This may represent novel sequelae of NTHi infection.