Rory McConn Walsh

Primary middle-ear lymphoma in a child

The case of a five year old boy who presented with a lower motor neurone facial nerve palsy secondary to primary non-Hodgkins lymphoma (NHL) of the middle ear is discussed. Any child who presents with a facial nerve palsy and conductive hearing loss requires thorough evaluation to exclude the possibility of temporal bone malignancy.

Laryngocele and squamous cell carcinoma of the larynx

We present a case report of a lady with a laryngocele and a squamous cell carcinoma of the larynx. The pathogenesis of the relationship between these two entities is discussed and the literature reviewed. This association means a carcinoma must be outruled if a laryngocele is detected clinically or radiologically.

Atypical clinical presentations of vestibular schwannomas.

A significant number of patients with vestibular schwannomas present atypically, with none of the classical symptoms of unilateral sensorineural hearing loss, tinnitus, and/or dysequilibrium. The aim of this study is to highlight those patients with unusual clinical symptoms. Study Design: The clinical data of all patients who presented to the vestibular schwannoma clinic at Beaumont Hospital over the past 12 years was prospectively recorded in a computerized database. This paper reviews the atypical presenting symptoms. Results: Three hundred ninety-eight patients were included in this study. A total of 3.7% of patients presented with atypical symptoms only. Conclusion: A significant subgroup, 3.7% in our study, did not present with the audiovestibular symptoms classically associated with vestibular schwannoma. Clinician awareness of the atypical clinical symptoms may lead to earlier detection of these lesions.

Cavernous angioma of the internal auditory canal

Cavernous angiomas of the internal auditory canal are rare lesions. The authors present a case of a 29-year-old lady with multiple infratentorial cavernous angiomas, whose sister had previously undergone surgery for a similar supratentorial lesion. She initially presented with an acute brainstem haematoma, secondary to a pontine cavernous angioma. Three years later she developed progressive right-sided sensorineural hearing loss and facial nerve paresis due to an internal auditory canal lesion. This was removed via the translabyrinthine approach and was found to be a cavernous angioma. This report underlines the multiple and dynamic nature of familial cavernous angiomas, as well as the importance of follow up to determine whether new symptoms are due to the enlargement of known angiomas or the development of new ones. As far as the authors are aware, this is the first report describing a cavernous angioma of the internal auditory canal in the context of familial and multiple infratentorial angiomas.

Supralabyrinthine approach to petrosal cholesteatoma

Petrosal cholesteatomas are rare lesions, which may be congenital or acquired in nature. We report an exceptional case occurring in a seven-year old girl who presented with a unilateral conductive hearing loss, despite normal tympanic membrane appearance. Early diagnosis was facilitated by computed tomography (CT) scanning. Although this case satisfied the criteria for congenital cholesteatoma, it is likely that the petrous apex was secondarily involved. Complete cholesteatoma removal was accomplished using a transtemporal supralabyrinthine approach, which allowed for hearing preservation, while avoiding the morbidity associated with a craniotomy. The present case constitutes the youngest case of petrosal cholesteatoma reported. We suggest that a lower threshold for the use of CT scanning in unilateral conductive hearing loss may allow for the earlier detection of more cases of petrosal cholesteatomas, as well as facilitating their removal using more limited approaches associated with less morbidity.

Computerized tomography is not reliable in the diagnosis of brainstem infection

The case of a 17-year-old girl who presented with a two-day history of absolute dysphagia secondary to a bulbar palsy due to a pre-pontine abscess is described. Rigid oesophagoscopy was normal and a neurology consultation suggested a central cause for her dysphagia. However the diagnosis was delayed because a computed tomography (CT) scan of her brain and brainstem was reported as normal. A subsequent magnetic resonance image (MRI) scan revealed a pre-pontine abscess. CT scanning is not as reliable as MRI in the diagnosis of infective lesions of the brainstem/brain, especially early in the course of the infection.

Presumed reduction of vestibular function in unilateral Menière's disease with aminoglycoside eardrops

We report the first case in the world literature of deliberate ablation of vestibular function in unilateral Menières disease with aminoglycoside eardrops (Garasone). These findings give conclusive proof that Garasone eardrops, and by implication, all topical aminoglycoside ear drops can be vestibulotoxic in the presence of a tympanic membrane defect. The lower concentration of gentamicin in Garasone, in comparison to the standard intratympanic preparations for chemical ablation of vestibular function, may also offer a reduction in the potential risk of cochleotoxocity.

Signs and Symptoms of Acoustic Neuroma at Initial Presentation: An Exploratory Analysis

Introduction The objective of this study was to describe the most common clinical features associated with an acoustic neuroma diagnosis and to identify those features associated with larger tumour size at initial diagnosis. Methods The clinical information of 945 consecutive patients diagnosed with acoustic neuroma at a single centre between 1992 and 2015 was analysed. Clinical features were examined and the relationship between these features and tumour size (>2.5 cm) was analysed using descriptive statistics and logistic regression analysis. Statistical analysis was performed in R version 3.1.1. Results The most common presenting symptom was a unilateral hearing loss in 752 patients (80%), with a progressive pattern in 90% of these cases. The second most common presenting symptom was unilateral tinnitus, accounting for 6.3%, while ataxia, vertigo and headache accounted for 3.8%, 3.4% and 2%, respectively. The diagnosis of acoustic neuroma was an incidental finding in 20 patients (2.1%). Temporal analysis demonstrated a downward trend in the number of patients presenting with hearing loss and an increased proportion of patients presenting with other symptoms. On multivariate analysis, larger tumour size was associated with abnormal tandem gait (odds ratio 8.9, p=0.02), subjective facial weakness (odds ratio 5.3, p< 0.001), abnormal facial sensation on examination (odds ratio 3.0, p=0.03) and headache (odds ratio 2.6, p< 0.001). Conclusion The majority of patients with acoustic neuroma present with the classic, progressive, unilateral hearing loss. However, the pattern of presentation in acoustic neuroma patients is changing. Features in the history indicative of a larger tumour are headaches and subjective facial weakness, whilst concerning features on examination are abnormal tandem gait and altered facial sensation.

Spontaneous CSF otorrhea from a defect in the medial wall of the middle ear.

A 50-year-old female patient presented with a 4-year history of recurrent right middle ear effusion. She had a ventilating tube inserted 4 years ago at another hospital, following which she did not report significant clear otorrhea. Subsequently she had a myringotomy 3 months ago. On this occasion she was noted to have pulsating clear fluid from the myringotomy site, which was confirmed to be CSF on -2 transferrin testing. MRI and CT showed opacification of the middle ear and mastoid air cells, and a pneumatized temporal bone, but the tegmen appeared intact. She was referred to the National Neuro-otology unit and underwent a combined approach tympanoplasty, which revealed a CSF-filled mucosal herniation arising in the medial wall of the middle ear (Fig 1). This filled the middle ear cavity, completely enveloping the ossicles, and herniated down the eustachian tube. No defect was found in the tegmen. The cyst was completely excised at its base and the middle ear, mastoid cavity, and eustachian tube were sealed with bone wax, fascia lata, temporalis muscle, and fibrin glue. An elective lumbar drain was inserted for 6 days. Postoperative recovery was uneventful

Our experience with 500 patients with benign paroxysmal positional vertigo: Reexploring aetiology and reevaluating MRI investigation

OBJECTIVE To explore the aetiology of and to evaluate the importance of MRI investigation on the posterior semicircular canal benign paroxysmal positional vertigo in an Irish population. METHODS A retrospective observational study of 500 patients with posterior semicircular canal benign paroxysmal positional vertigo, diagnosed and treated by the senior author over a 10-year period. Most patients underwent an MRI brain and inner ear, following the same scan protocol. This included T1 weighted sagittal IR-FSPGR volume, axial T2 weighted, gradient echo T2 weighted and FLAIR sequences plus time of flight cerebral angiography. RESULTS The average age of presentation was 56 years; with the overall female to male ratio was 1.6:1, which was largely the net results of 2 age groups. Over 30% of our patients recalled distinct aetiological triggers, of which the top 3 were trauma, infection, and surgery. These accounted for 16%, 6%, and 5%, respectively. More than 25% of the patients were discovered to have abnormal intracranial findings on MRI. The 2 most common non-infarct incidental findings were neoplasia and vascular abnormalities. Although fewer than 20 patients had acute intracranial haemorrhage or malignant tumours, most of them were urgently referred to neurosurgeon due to the life-threatening nature of the condition. One round of particle repositioning manoeuver was successful in treating 84% of the patients, and the 2-year recurrence rate was only 2.2%. CONCLUSION The diagnosis of posterior semicircular canal benign paroxysmal positional vertigo is thought to be relatively easy to make, and the treatment is highly effective. Clinicians should be fully aware of and prepared for the diverse aetiology, and thus have no hesitation in requesting MRI scan as an important investigation.