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Hearing preservation post resection of grade 3 endolymphatic sac tumour (ELST) via a translabyrinthine approach: A First Report
AA Esmaili*, GP Rajan*
* Department of Otolaryngology Head and Neck Surgery, Fiona Stanley Hospital
Introduction:
Endolymphatic sac tumours (ELSTs) are slowly growing, benign but locally malignant neoplasms arising from the epithelium of the endolymphatic system. They may either be sporadic or associated with Von Hippel-Lindau syndrome (VHL).(1) Sensorineural hearing loss (SNHL) can affect greater than 90% of afflicted patients.(1) Hearing preservation surgery is undertaken when tumours are small. Grade 3 tumours frequently are removed by a trans-labyrinthine approach resulting in a dead ear. Intratympanic corticosteroids exhibit otoprotective effects through various pathways.(2) This effect may be utilised to preserve hearing when a trans-labyrinthine approach is used.
Aims:
We present a case of a patient with VHL associated grade 3 ELST whom underwent trans-labyrinthine resection with preservation of preoperative hearing levels. Preoperative and perioperative intratympanic corticosteroids were utilised for otoprotection.
Methods:
The case was analysed and presented. A pubmed search for the term "endolymphatic sac tumour" was performed. Research articles and case reports presenting clinically features of patients prior to and following resection of ELSTs were reviewed.
Results:
Following resection hearing preservation was demonstrated. To our knowledge this is the first time this has been reported following a trans-labrynthine approach. We hypothesise that steroids played a role in hearing preservation. A common feature of the literature review was that hearing outcomes postoperatively were not reported.
Conclusion:
Endolymphatic sac tumours are uncommon and when present can be associated with VHL. In such patients they occur more commonly in women and are more frequently bilateral.(1) Following trans-labrynthine approach for resection, a dead ear is the rule. As demonstrated, pre-operative intra-tympanic steroids may be utilised to achieve hearing preservation during translabyrinthine approaches. In larger scale studies post operative hearing status is often omitted. Post operative hearing status should be documented in order to attempt to establish hearing preservation techniques and protocols after labyrinthectomies and translabyrinthine approaches.
References:
1. Bambakidis NC, Megerian CA, Ratcheson RA. Differential grading of endolymphatic sac tumor extension by virtue of von Hippel-Lindau disease status. Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology. 2004;25(5):773-81. Epub 2004/09/09. 2. Lavigne P, Lavigne F, Saliba I. Intratympanic corticosteroids injections: a systematic review of literature. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery. 2015. Epub 2015/06/24.
Cervical sympathetic chain paraganglioma as a rare cause of unilateral tonsillar enlargement
A A Esmaili*, M Gilfillan*, R Wormald*
* Department of Otolaryngology Head and Neck Surgery, Fiona Stanley Hospital, Western Australia
Introduction:
Unilateral tonsillar enlargement (UTE) is a common presentation to the otolaryngologist. Malignancy is the major concern and bilateral tonsillectomy is often undertaken to disprove this. Careful examination of the clinical features may help guide selection of patients for tonsillectomy and identify those that may require further pre-operative investigations including imaging.
Aims:
We report a rare case of 30 year old female with a sympathetic paraganglioma presenting initially as unilateral tonsillar enlargement. While awaiting tonsillectomy patient developed palpitations, sweats and tremors. Imaging of the neck revealed a paraganglioma extending into the left oropharynx. Tonsillectomy in this patient may have led to a catastrophe.
Methods:
A retrospective case review was undertaken. Data on the clinical features on presentation, imaging and post operative histopathology was collated and presented. A pubmed search for "paraganglioma" and "unilateral tonsillar enlargement" was undertaken and research articles considering clinical assessment and management of these conditions were reviewed.
Results:
A paraganglioma is a very rare cause of UTE. If inadvertently treated with tonsillectomy it may lead to an intra operative catastrophe. Appropriate history and examination together with imaging is the key to this diagnosis. Latest research argues against systemic tonsillectomy in isolated UTE.1 Imaging may be required in patients with co-existing systemic symptoms pointing to an alternative cause.
Conclusion:
This case highlights the importance of careful clinical history and examination in UTE which may reveal co-existing symptoms that may indicate parapharyngeal tumour as a cause. In such situation imaging in UTE is indicated. Furthermore, we advocate better patient selection for progression to tonsillectomy in isolated UTE based on clinical assessment.
References:
1. Berkowitz RG, Mahadevan M. Unilateral tonsillar enlargement and tonsillar lymphoma in children. The Annals of otology, rhinology, and laryngology. 1999;108(9):876-9. Epub 1999/10/20.
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