Egyptian Journal of Ear, Nose, Throat and Allied Sciences xxx (2016) xxx–xxx Contents lists available at ScienceDirect Egyptian Journal of Ear, Nose, Throat and Allied Sciences journal homepage: www.ejentas.com Case report Primary Hodgkin’s lymphoma of the middle ear: A rare cause of facial nerve palsy N Maithrea a,b,⇑, S Periyathamby a, Irfan Mohamad b a b Department of Otorhinolaryngology-Head and Neck Surgery, Penang General Hospital, Jalan Residensi, 10900 Penang, Malaysia Department of Otorhinolaryngology-Head and Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia Health Campus, 16150 Kota Bharu, Kelantan, Malaysia a r t i c l e i n f o Article history: Received 11 October 2016 Accepted 30 October 2016 Available online xxxx Keywords: Hodgkins lymphoma Middle ear Facial nerve palsy a b s t r a c t Facial nerve palsy can occur whenever any part of the facial nerve is affected It can be complete or partial, unilateral or bilateral and upper motor or lower motor neurone type Common causes of unilateral lower motor neuron facial nerve palsy include trauma, infections of the middle ear, neoplasms of the parotid, iatrogenic and idiopathic Hodgkin lymphoma typically presents with an asymptomatic lymphadenopathy, but associated symptoms include constitutional symptoms, intermittent fever, chest pain, or shortness of breath We present an extremely rare case of facial nerve palsy secondary to primary classical nodular sclerosis Hodgkin’s lymphoma, arising from the left middle ear extending into the external auditory canal Ó 2016 Egyptian Society of Ear, Nose, Throat and Allied Sciences Production and hosting by Elsevier B.V This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-ncnd/4.0/) Introduction Lymphomas are the commonest non-epithelial malignancies of the head and neck.1 However, these lymphoreticular neoplasms seldom present in the temporal bone area unless in cases of acquired immunodeficiency syndrome.2 Temporal bone lymphomas are typically metastatic in nature or spread from contiguous foci.3 Sporadic cases have been reported in literature, and sites of origin include the middle ear cleft, mastoid, and external auditory canals.4,5 Case report A 53-year-old Indian male presented with one month history of left-sided lower motor neuron facial nerve palsy associated with left-sided hearing loss He was treated initially with a combination of antibiotic and steroid therapy by a private practitioner However, the condition did not improve On examination he had a left-sided lower motor neurone facial nerve palsy (Grade III House-Brackmann classification) A mass was visible filling the ⇑ Corresponding author at: Department of Otorhinolaryngology-Head and Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia Health Campus, 16150 Kota Bharu, Kelantan, Malaysia E-mail address: maithrea1@gmail.com (N Maithrea) Peer review under responsibility of Egyptian Society of Ear, Nose, Throat and Allied Sciences external ear canal Tuning fork test confirmed the hearing loss, which was conductive in nature The remainder of the physical examination was normal Blood tests were normal High resolution contrast enhanced computed tomography (CT) scan of the brain and temporal bones showed an irregular hypodense lesion in the left middle ear cavity and left external auditory canal Bony erosion of left ossicles, mastoid and tegmen tympani was noted with dehiscence of left facial nerve canal (Fig 1) There was no intracranial extension He underwent exploration of his left ear and mastoid A pale fleshy mass of tissue occupied the epitympanum of the middle-ear cleft, external auditory canal and mastoid bowl The dura was not breached Histopathological analysis showed a nodular growth pattern with areas of necrosis (Fig 2) The nodules were composed of a mixed population of cells including small lymphocytes, eosinophils, histiocytes and a moderate number of classical, lacunar and occasionally mummified Reed-Sternberg cells Immunohistochemical studies were positive for CD15 and CD30 (Figs and 4), and negative to ALK-1 Impression was classical nodular sclerosis Hodgkin’s Lymphoma and staging investigations were done, including bone marrow aspirate, and CT neck/abdomen/thorax which revealed no other focus He was commenced on a cycle regime of ABVD (Adriamycin, Bleomycin, Vinblastine and Dacarbazine) chemotherapy with a FDG PET scan planned after completion to assess response After cycles of chemotherapy his facial nerve palsy was noted to have resolved completely, with repeat pure tone audiometry planned after completion of treatment http://dx.doi.org/10.1016/j.ejenta.2016.10.010 2090-0740/Ó 2016 Egyptian Society of Ear, Nose, Throat and Allied Sciences Production and hosting by Elsevier B.V This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/) Please cite this article in press as: Maithrea N., et al Primary Hodgkin’s lymphoma of the middle ear: A rare cause of facial nerve palsy Egypt J Ear Nose Throat Allied Sci (2016), http://dx.doi.org/10.1016/j.ejenta.2016.10.010 N Maithrea et al / Egyptian Journal of Ear, Nose, Throat and Allied Sciences xxx (2016) xxx–xxx Fig positive immunohistochemical staining for CD 30 Fig Irregular hypodense lesion in the left middle ear cavity with ossicular erosion Fig Nodular growth pattern with broad collagen bands surrounding nodules Fig positive immunohistochemical staining for CD 15 the roof, the jugular wall as the floor, the tympanic membrane as the lateral wall and oval window as the medial wall Anteriorly it is bound by a thin layer of bone that separates the carotid canal and the tympanic cavity called the carotid wall, and posteriorly it contains the mastoid antrum, communicating with the mastoid air cells.6 It contains the ossicles, important musculature including the stapedius, the facial nerve, vascular supply and lymphoid tissue, which drains into the retropharyngeal and deep cervical group of lymph nodes.7 The facial nerve traverses the tympanic cavity in a bony canal on the labyrinthine wall just the footplate of the stapes It enters the temporal bone at the internal auditory meatus Here it passes laterally and bends backwards and passes in the Fallopian canal above the oval window until it reaches the aditus where it turns downwards and passes vertically until it reaches the stylomastoid foramen and exits out of the temporal bone.8 Lymphomas most commonly present as asymptomatic painless lymphadenopathy, with 60–80% occurring in the cervical and supraclavicular nodes, with other common areas being in the axilla and the inguinal nodes However, in our case, the patient did not have any palpable lymph nodes, and CT scan done also did not show any lymph node enlargement A large proportion of patients develop systemic symptoms, especially B symptoms like fever, weight loss and night sweats before the finding of lymphadenopathy, present in up to 50% of those with advanced disease These symptoms were also absent in our patient Hodgkins lymphoma typically starts from a single foci of lymphatic tissue, and via lymphatic channels, spread to adjacent lymph nodes and finally disseminating to distant non adjacent sites and organs While lymphomas are the second most common malignancies of the head and neck, lymphomas in the middle ear are relatively rare.10 Literature review shows that there has only been 18 reported cases of lymphomas in the ear since 1947.11 Nodular sclerosis Hodgkins lymphoma is the most common subtype of Hodgkin’s lymphoma, accounting for 60–80% of cases.12 Nevertheless, the subtype of lymphoma does not influence treatment modality as much as the stage of the disease In this patient, there was only involvement of one group of lymph node with contiguous extranodal involvement, and prognosis for low grade HL is excellent, with cure rates of up to 90% Even in high risk HL, cure rates are achieved in up to 80% with a multimodal approach of chemotherapy and radiotherapy.13 Discussion Conclusion The middle ear cleft is located at the petrous part of the temporal bone and extends from the tympanic membrane to the cochlea It is often described as a six-sided box with the tegmental wall as Malignancy should always be excluded in a setting of therapyresistant otitis media or lower motor neuron facial paralysis The Please cite this article in press as: Maithrea N., et al Primary Hodgkin’s lymphoma of the middle ear: A rare cause of facial nerve palsy Egypt J Ear Nose Throat Allied Sci (2016), http://dx.doi.org/10.1016/j.ejenta.2016.10.010 N Maithrea et al / Egyptian Journal of Ear, Nose, Throat and Allied Sciences xxx (2016) xxx–xxx atypical presentation and rare location of this case of Hodgkin’s lymphoma also highlights the value of correlation of underlying anatomy to the lesion site in order to guide further investigation and management References DePena CA, Van Tassel 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enters the temporal bone at the internal auditory meatus Here it passes laterally and bends backwards and passes in the Fallopian canal above the oval... should always be excluded in a setting of therapyresistant otitis media or lower motor neuron facial paralysis The Please cite this article in press as: Maithrea N., et al Primary Hodgkin? ? ?s lymphoma. .. trial (ISRCTN97144519) J Clin Oncol 2010;28(20):3352–3359 Please cite this article in press as: Maithrea N., et al Primary Hodgkin? ? ?s lymphoma of the middle ear: A rare cause of facial nerve palsy